CORD-19:775b90287b44353bee979abb0970a4666022f86d JSONTXT

ABSTRACTS PRESENTED AT CONCURRENT SESSIONS ATYPICAL PRESENTATION OF HONEY BEE STING HYPER- SENSITIVITY Abstract Introduction: A bee sting can result in a myriad of manifestations ranging from acute localized cutaneous response to unusual delayed reactions. Here we report a case of a 15 year old boy who developed bloody diarrhea 72 hours following a bee sting. To the best of our knowledge this is the first such report of delayed gastrointestinal manifestation. Case Report: A 15 year old male presented for evaluation of allergy to honey bee. He stated that on 2 occasions he was stung by a bee resulting in localized swelling of affected area, nausea, vomiting and abdominal pain after the sting, then bloody diarrhea approximately 72 hours after. The most recent episode was 1 week prior to clinic visit when the patient was stung by a bee on his toe, following which he had a local reaction. Then 3 days later he had abdominal pain and loose stools with blood. Examination revealed a well healed puncture mark on his toe. His vitals were normal and CBC, ESR, CMP and a stool culture were unremarkable. Skin prick testing was positive for honey bees. Intradermal testing at lowest concentration (1/100000) was positive for all other Hymenoptera (wasp, yellow jacket, hornet) with negative saline. Immunocap showed positivity to all Hymenoptera species. Discussion: Delayed gastrointestinal hemorrhage is an unusual manifestation after sting by a honey bee that cannot be explained by IgE-mediated mechanisms. We postulate that this response could be due to a delayed type hypersensitivity leading to deposition of immune complexes in the basement membrane of small blood vessels causing vasculitis. This may explain the delayed manifestation of bloody diarrhea 72 hours after the sting. It is also possible that the etiology of this unusual reaction may not be immunologically mediated and may be due to a direct cytotoxic effect of honey bee venom component like Melittin on gastrointestinal tissue. Melittin is known to cause complement cleavage and the release of Bradykinin which are both associated with hemolysis. Studies indicate that Melittin and Phospholipase A2 can decrease smooth muscular contractility and thus potentially induce hemorrhage. Conclusion: We report an unusual and rare case of bee sting induced bloody diarrhea in an adolescent which was reproducible on two different occasions. We hypothesize that this could be due to a delayed hypersensitivity reaction or a direct cytotoxic effect of bee venom. S. Melethil * , S. Ho, R.S. Mehta, Galveston, TX. Introduction: A bee sting can result in a myriad of manifestations ranging from acute localized cutaneous response to unusual delayed reactions. Here we report a case of a 15 year old boy who developed bloody diarrhea 72 hours following a bee sting. To the best of our knowledge this is the first such report of delayed gastrointestinal manifestation. Case Report: A 15 year old male presented for evaluation of allergy to honey bee. He stated that on 2 occasions he was stung by a bee resulting in localized swelling of affected area, nausea, vomiting and abdominal pain after the sting, then bloody diarrhea approximately 72 hours after. The most recent episode was 1 week prior to clinic visit when the patient was stung by a bee on his toe, following which he had a local reaction. Then 3 days later he had abdominal pain and loose stools with blood. Examination revealed a well healed puncture mark on his toe. His vitals were normal and CBC, ESR, CMP and a stool culture were unremarkable. Skin prick testing was positive for honey bees. Intradermal testing at lowest concentration (1/100000) was positive for all other Hymenoptera (wasp, yellow jacket, hornet) with negative saline. Immunocap showed positivity to all Hymenoptera species. Discussion: Delayed gastrointestinal hemorrhage is an unusual manifestation after sting by a honey bee that cannot be explained by IgE-mediated mechanisms. We postulate that this response could be due to a delayed type hypersensitivity leading to deposition of immune complexes in the basement membrane of small blood vessels causing vasculitis. This may explain the delayed manifestation of bloody diarrhea 72 hours after the sting. It is also possible that the etiology of this unusual reaction may not be immunologically mediated and may be due to a direct cytotoxic effect of honey bee venom component like Melittin on gastrointestinal tissue. Melittin is known to cause complement cleavage and the release of Bradykinin which are both associated with hemolysis. Studies indicate that Melittin and Phospholipase A2 can decrease smooth muscular contractility and thus potentially induce hemorrhage. Conclusion: We report an unusual and rare case of bee sting induced bloody diarrhea in an adolescent which was reproducible on two different occasions. We hypothesize that this could be due to a delayed hypersensitivity reaction or a direct cytotoxic effect of bee venom. Background: Early, massive exposure to milk allergens is traditionally considered a risk factor for cow's milk allergy (CMA) . Epidemiological studies, however, beg the question whether it can be protective; high milk allergen exposure has also been hypothesized as policy for food allergy prevention. In this context, short bowel syndrome has been anecdotally associated with CMA, and no studies have used confirmatory milk challenges. Methods: A review of consecutive patients followed with short bowel syndrome over a ten-year period at a tertiary pediatric institution. Patients included only the patients who fulfilled the following criteria: -Intestinal failure following SBS, defined as dependency on parenteral nutrition providing at least 50% of the total required intake for at least three months -Clinical follow-up from surgery to the complete introduction of a diversified diet including cow's milk proteins. Infants with symptoms of CMA underwent a milk-specific IgE sensitization test and a confirmatory oral milk challenge. Results: Three infants in a caseload of 32 eligible patients were confirmed with CMA at challenge (see Table) . This 9.4% proportion represents two to ten-folds the incidence of CMA in open populations. Conclusion: With its 9.4% incidence, our caseload of SBS children carry a two-to tenfold risk of CMA compared to open populations. This suggests that a low digestive capacity may play a role in the high incidence of food allergy in children. As their peptic digestion is not complete during early life, protein remnants of the diet could act as allergens. If the overexposure to milk allergens is among the causes of CMA in these children, our results warn against the hypothesis of massive antigen exposure as instrument of food allergy prevention. Characteristics of the caseload of infants with SBS Background: Resistance to antibiotics is a serious worldwide problem. When antibiotic choices are limited due to resistance, treatment alternatives for resistant infections may have higher toxicity, be more costly, and be less effective, and thus patients with resistant infections have higher morbidity and mortality. Core methods to prevent antibiotic resistance include the appropriate use and choice of antibiotics. Objective: To reduce the use of prophylactic vancomycin, levofloxacin, and clindamycin in patients with a history of penicillin allergy undergoing surgery by implementing a new clinical pathway consisting of allergy consultation and penicillin allergy skin testing as part of the preoperative evaluation clinic visit. Methods: The participants in this clinical practice improvement project were patients with a history of penicillin allergy who were scheduled to undergo surgery and referred by the Preoperative Evaluation Clinic team for allergy consultation and penicillin allergy skin testing from August 2012 to August 2013, the first year of this new clinical pathway. The primary outcomes were the percent reductions in the use of prophylactic vancomycin, levofloxacin, and clindamycin in patients with a history of penicillin allergy when compared to historical controls. Results: Of the 384 patients with a history of penicillin allergy who underwent allergy consultation and penicillin allergy skin testing, 360 (94%) had negative penicillin allergy skin test results and were given clearance by the allergist to receive penicillin or cephalosporin antibiotics. For patients who had reported penicillin allergy, within six months of implementation of this new clinical pathway, vancomycin use was reduced by 54%, levofloxacin use was reduced by 40%, and clindamycin use was reduced by 22% compared to historical controls. Conclusions: Prophylactic use of vancomycin, levofloxacin, and clindamycin in patients with a history of penicillin allergy undergoing surgery can be reduced by allergy consultation and penicillin allergy skin testing during the preoperative evaluation process. S.B. Sindher * , S.P. DaVeiga, Philadelphia, PA. Introduction: Most fruit allergies manifest as pollen-food allergy syndrome characterized by pruritus and edema of the lips, tongue, palate and throat usually a few minutes after ingestion of the raw fruit. Orange is among fruits that are associated with pollen-food allergy syndrome but rarely causes anaphylaxis. Methods: We review the case of a 31-month-old girl who developed severe anaphylaxis after the consumption of one Cutie® orange. Results: A 31-monthold female presented in anaphylactic shock to an emergency department (ED). Prior to presentation, she was at a supermarket, ate one Cutie® orange and was playing with the orange peel. Within a few minutes she had bilateral periorbital swelling and pruritis. She was treated with diphenhydramine at the supermarket. The patient developed lip and tongue swelling and respiratory distress so her parents drove her to the ED within 10 minutes. Examination was notable for a pulse oximetry reading of 79% on room air, diffuse wheezing in all lung fields, lip and tongue angioedema and hives. She was treated with nebulized albuterol, IV diphenhydramine, ranitidine, 2 doses of subcutaneous epinephrine, methylprednisolone and an IV bolus of normal saline. She continued to have worsening respiratory distress and hypoxia and subsequently required intubation; she was noted to have significant upper airway edema on intubation. After intubation she was transferred via a helicopter to a Pediatric Intensive Care Unit. A chest x-ray was performed to check endotracheal tube placement and revealed right upper lobe atelectasis and pulmonary edema ( Figure 1 ). Her symptoms improved overnight and she was discharged home after a 48hour hospitalization. Her past medical history was notable for a varied diet and prior consumption of orange juice without reaction. She had reported nightly cough at baseline with cough on exertion but was not on therapy for asthma. In follow up at Allergy/Immunology as a new patient, she had a positive skin prick test result to orange and peach. She was advised to avoid orange and peach and also started on asthma therapy. Conclusion: We present the first case report of near-fatal anaphylaxis to orange in a toddler. This case confirms that poorly controlled asthma is a risk factor for difficult to treat IgE-mediated reactions to foods, including orange. Background: Food protein-induced enterocolitis syndrome (FPIES) usually presents in infancy. In the acute form, when food is ingested on an intermittent basis or following a period of avoidance, FPIES typically presents with profuse vomiting, diarrhea, and dehydration, starting 1-3h following specific food ingestion. FPIES diagnosis is based on history and typical symptoms that improve with food avoidance, and with the exclusion of other etiologies. Oral food challenge (OFC) remains the gold standard for diagnosis. Case: A 6-month-old female infant was referred for FPIES of difficult management. The child was admitted to the hospital at 6 weeks of life for suspected sepsis occurred 15 days after receiving milk thickened with rice cream; sepsis evaluations were negative. At 5 months, soon after her first rice-containing meal, she developped emesis, pallor, followed by diarrhea and lethargy, so she was hospitalized for suspected anaphylaxis. Negative allergy tests (SPT, ImmunoCAP for rice, milk, egg, wheat) and the clinical history indicated the diagnosis of rice-induced FPIES. For the investigation of cereal tolerance she was challenged with commercial maize to which she reacted with vomiting, hypotonia and bloody diarrhea. Industrial maize food contamination with rice was suspected. We requested a pure whole wheat flour from Heinz Baby Food, Italy ® , which was tolerated at challenge. A special diet was initiated, in col-laboration with Heinz, using the same rice-free flour to make pasta to which she did not react. Introduction of solids was predicated on a series of OFCs, including wheat, beef, pork, chicken, potato, carrot, spinach, zucchini, tomatoes which were negative. However, each introduction of foods contaminated with rice determined the characteristic symptoms of the syndrome. In one occasion, a typical FPIES reaction followed the administration of mauve suppositories containing rice starch. Conclusion: This case highlights how FPIES onset can be very early and how tiny amount of food can be enough to provoke symptoms, reflecting the degree of hypersensitivity in individual patients. Early recognition of this condition and the management with cross-contaminant-free baby foods are crucial in preventing recurrences. The co-operation among pediatricians and industry is of outstanding importance. A. Barbir *1 , M. Janelli 1 , M. Lin 1 , R.A. Wolf 2 , J. Dennerlein 1 , 1. Boston, MA; 2. Plantation, FL. Introduction: Various auto-injectors differ in their form factor, mechanism of drug delivery, and user instructions, which can affect the successful use of the device through applied force capability and consistency of device orientation during drug delivery. We hypothesized that (1) a device's form factor will impact users' capability of applying force as measured by the maximum applied force and (2) a device's form factor, mechanism, and instruction will impact motor control performance metrics. Methodology: Trainer devices of 3 commercially available epinephrine auto-injectors with 3 different form factors (cylindrical, elliptical, and prismatic) were tested in a laboratory-based repeated measures experiment with 20 adult (aged 18-30 years) participants. Participants performed 2 tasks using a power grip with each of the 3 injectors: a maximum force capability task in which they applied their maximum possible force onto a force plate positioned over their thigh and an application task in which, after watching the device's training video, they practiced an injection using the trainer. For the application task, device performance included time to hold force, force, and device orientation and its variability during the hold segment. Participants rated their force confidence and preference for the 3 devices. Results: Greatest force capability was exhibited by the device with the elliptical form factor followed by the prismatic and then the cylindrical, with the difference between the elliptical and the cylindrical form factors being statistically significant. For the application task, the elliptical form factor device had the fastest time to force. The prismatic form factor device had the largest angle and variability in the angle of the resultant applied force. Participants reported the highest force confidence when using the elliptical and cylindrical form factor devices, ranking the elliptical as their preferred device. Conclusion: The results suggest that the elliptical form factor may have better success in drug delivery in a larger set of the population. Table 1 Parameters measured for (I) maximum force capability task, (II) application task, and (III) self-reported survey. For significant main effects, Tukey's post hoc groupings are ranked such that A > B. Values with the same superscript letters indicate no significant difference. uary 1, 1993 1, , through March 31, 2014 PubMed, Medline, CENTRAL, EMBASE, Scopus, ISI, Regulatory Documents, Web of Science and manufacturers' web clinical trial registries (GlaxoSmithKline and AstraZeneca) with the clinical trial filters using multiple search terms with no language restrictions. We included all studies that compared patients 14 years of age and older on ICS and not on ICS relative to the risk of incident pneumonia (community acquired, lower respiratory tract infection, non-tuberculous mycobacterial pneumonia). We then summarized individual study estimates into two random-effect meta-analyses, one including randomized controlled trials (RCTs) and another one including observational studies (OBS). Results: There were total of 12 studies, 7 RCTs including total of 10,585 and 5 OBS including total of 44,127 participants. There was no heterogeneity in RCTs and summarized estimated effect of ICS was protective of pneumonia; OR 0.75 (0.57-0.99. p=0.05). On the contrary, OBS showed moderate heterogeneity (I 2 =48%) with resulting summed OR of 1.94 (1.42-2.65, p<0.0001), suggesting increased risk of pneumonia with use of ICS in asthma patients. However, OBS had lower grade of confidence compared to RCTs. Conclusions: ICS may carry protective effect or at the very least do not have increased risk of incident pneumonia in patients with asthma, based on our meta-analysis of available RCTs. While observational studies suggested higher risk of pneumonia in similar patients, the observed heterogeneity and inherent methodological limitations confered lower grade of confidence in these studies. Meta-analysis of RCTs and OBS for pneumonia (Risk estimates shown are odds Ratio (OR) for RCTs and OBS. H. Li *1 , A. Reshef 2 , H. Farkas 3 , J. Baker 4 , G. Porebski 5 , D. McNeil 6 , A. Relan 7 , A. Zanichelli 8 , 1. Chevy Chase, MD; 2. Tel Hashomer, Israel; 3. Budapest, Hungary; 4. Lake Oswego, OR; 5. Krakow, Poland; 6. Columbus, OH; 7. Leiden, Netherlands; 8. Milan, Italy. Background: Hereditary angioedema (HAE) due to C1 inhibitor deficiency is characterized by recurrent episodes of disabling and painful tissue swelling. Although some attacks are self-limiting and may resolve spontaneously, many attacks progress before taking several days to improve. We reviewed the efficacy of recombinant human C1 inhibitor (rhC1INH) in the treatment of severe HAE attacks in a randomized controlled study. Methods: Seventy-five HAE patients were randomized (3:2) to receive 50 IU/kg of rhC1INH or saline in a multi-center double-blind study. Severity of attack symptoms was assessed at baseline using a 100mm visual analog scale (VAS). Patients could be randomized if the baseline VAS score was ≥50mm. Severe attacks were defined as those with a baseline VAS score ≥75mm. The primary endpoint was time to onset of symptom relief, assessed as time from start of study drug infusion to onset of sustained beneficial effect, defined by patient responses demonstrating improved symptoms on a Treatment Effects Questionnaire (TEQ). Rescue treatment with open-label rhC1INH was permitted if there was no relief of symptoms by 4 hours or in case of life-threatening symptoms; these attacks were censored in the primary analysis. IRB approval and written informed con-sent was obtained from all patients. Results: Among the 75 randomized patients, the median VAS score at baseline was 76mm. Forty-three patients were determined to have severe attacks (rhC1INH: 24; saline: 19). Among patients with severe attacks, the median (95% CI) time to onset of symptom relief in rhC1INH-and saline-treated patients respectively, was 90 min (47, 120) versus 334 min (105, not estimable); hazard ratio 2.52, log rank p-value 0.02. The proportion of patients with severe attacks who received open-label rhC1INH as rescue medication was 1/24 (4%) in the rhC1INH group and 10/19 (53%) in the saline group. After receiving rescue with rhC1INH, saline-treated patients with severe attacks had a median time to relief of 60 min, as measured from the time of rescue administration. Conclusions: rhC1INH was effective in resolving severe HAE attacks. Patients randomized to saline who had worsening or sustained angioedema symptoms had rapid improvement following rescue with rhC1INH. J.J. Anderson * , P. Pityn, London, ON, Canada. Introduction: The long harsh winter of 2013-14 prompted allergy experts to predict a "pollen vortex". They reasoned that delayed antithesis would result in the sudden release of greater than normal amounts of tree pollen. We evaluated the actual tree pollen data for London Ontario (Canada) to determine if experts had predicted correctly. Methods: Daily samples of airborne pollen were collected during the 2014 tree season with a Burkard spore trap located 3 stories above grade. The samples were analyzed and compared to our historical 12 year data. The pollen counts were normalized against the 12 year average count for each of the respective pollen types to assess the 2014 tree season. The total tree pollen count and preponderance of 15 individual tree pollen types were examined, along with the start and duration of their pollination. Results: Contrary to the experts' predictions, the total seasonal pollen count was not elevated, but was down considerably and several individual tree pollen levels were far lower than at any other time in the previous 12 years. Specifically, pollen levels of maple, juniper, birch, ash, mulberry and walnut were considerably lower, as much as 4-5 times lower than the average. The other tree pollen counts were within normal range. Conclusions: Not only did the forecasted pollen burst fail to materialize this year, but the exact opposite happened. In 2014, the total tree pollen counts were at historic lows for London, Ontario. Six major pollen producers of the fifteen tree types were at historic lows despite otherwise typical duration and start of their respective seasons. Experts need to refine their modelling skills to better predict pollen in the face of global climate change. Background and Purpose: The cyanobacteria specie, Microcystis aeruginosa (Ma), produces microcystin (MC) and an array of diverse metabolites believed responsible for their toxicity and/or immunogenicity. Previously, chronic rhinitis patients were demonstrated to elicit a specific IgE response to non-toxic strains of Ma by skin-prick testing (SPT) indicating that cyanobacteria allergenicity likely resides in the non-toxin producing component of the organism. The objective of this study was to investigate the functional interactions between cyanobacteria toxins and their co-expressed immunogenic peptides. Methods: Sera collected from chronic rhinitis patients previously found to be sensitized by SPT to Ma were used to identify sensitizing proteins. Direct and indirect IgE-specific ELISAs were used to confirm sensitization to Ma. A 2-D gel electrophoresis followed by specific IgE immunoblot and mass spectroscopy (MS) was performed to identify the relevant allergenic peptides. Cyto-toxicity and mediator release assays were performed using lysates from Ma toxic (MC+) and non-toxic strain (MC-) . Results: Using lysates from MC+ and MC-Ma strains and sera from Ma sensitized patients, specific IgE was increased in response to the MC-but not the MC+ strain. In addition, MC-extracts induced b-hexosaminidase release using rat basophil leukemia cells. 2-D gel electrophoresis followed by MS revealed the relevant sensitizing peptides were phycocyanin and the core-membrane linker peptide, both components of the cyanobacteria phycobilisome complex. Of note, the IgE specific response was dose-dependently inhibited by addition of microcystin to the MC-lysate. Conclusions: The allergenicity of Ma is inversely proportional to its microcystin content suggesting a direct or indirect interaction between microcystin toxin and the allergenic peptides. Further investigation is warranted to understand the interplay between immunogenicity and toxicity of cyanobacteria under diverse environmental conditions. D.A. Jara * , C.S. Barnes, J. Portnoy, M. Dhar, Kansas City, MO. Rationale: Fungus can be found outdoors and indoors and can enter the home through multiple mechanisms such as a window, on clothing, or openings in the home. Patients have shown a higher risk of atopy with fungal exposure. To determine if indoor mold count was elevated in relation to elevated outdoor mold counts we conducted the following study. Methods: Data was retrieved from the Kansas City mold and spore database for a 15 year period from March 1998 thru August 2013. The community wide outdoor spore count was performed with a with a Burkard device according to National Allergy Bureau Protocols. The indoor spore count data was collected from an Allergenco MK3 or a Buck Bioaire from about 150 homes with a mean of 4 collections taken from each home (Basement, Bedrooms, Living Room, Kitchen, Bathroom, etc) . Outdoor ground level data is the outdoor collection taken at each home at the time the indoor collections were taken. Data included here for comparative purposes is for 8 spore types that are easily identifiable individual genera. Data is reported by percent of collections in which each genera was observed. Spore counts were stored in an Access database and analyzed using Excel. Results: Total spore percentages are seen in table 1. Cladosporium was the most common spore collected indoors and outdoors. When comparing indoor counts, Cladosporium was most commonly seen in basement at 52% followed by child's bedroom 20%. Alterneria was most commonly seen in the child's bedroom at 30% followed by bathroom 19% and master bedroom 15%. Overall spores were most commonly seen in child's bedroom 16%, basement 13% and kitchen 10%. Alternaria was least commonly seen in family room 4% basement 9% and living room 9%. Cladosporium was least commonly seen in family room 2%, master bedroom 3% and living room 6%. Overall spores were least commonly seen in family room 1%, master bedroom 2% and living room 6%. Conclusion: Increased outdoor spore count is associated with higher indoor spore counts but origin could not be inferred from this study. Cladosporium was the most identified fungi both indoors and outdoors in this study. Interestingly the child's bedroom seems to be the most susceptible to indoor mold amplification, while the living room and family room had the lowest spore count. This may be secondary to the aesthetic desire most families put into maintaining a home while negating lesser used rooms. Table 1 Percentage of Total spore counts seen. V. Rodinkova 1 , A. Prikhodko 2 , A. Maleeva 2 , O. Palamarchuk 1 , I. Motruk 1 , L. Kremenska 1 , K. Musatova 1 , L.M. DuBuske *3 , 1. Vinnitsa, Ukraine; 2. Zaporizhzhia, Ukraine; 3. Gardner, MA. Background: Ambrosia is increasing in abundance in south and east Ukraine including Donetsk, Zaporizhzhia, Mykolaiv, Kherson, Kirovograd and Dnepropetrovsk regions Methods: Pollen collection was done by gravimetric sampling in Vinnitsa for years 1999 and 2000 . From 2009 to 2013 volumetric methods employing a Burkard trap placed at a height of 25 meters above the ground on the roof of a Vinnitsa Medical University building were used. Pollen counts in Zaporizhya from 2006 to 2013 and in Poltava, Donetsk, Dnepropetrovsk, Odessa and Simferopol in 2010 used volumetric Burkard spore traps sampling from the March 1 until October 1. Results: Greatest ragweed pollen counts were in the third ten-day period of August and for the first or second ten-day period of September, seen in Vinnitsa since 1999 and in Zaporizhya from 2006 until 2010 similar to all other cities in 2010. Ragweed pollen increase occurred in the third ten-day period of August being maximal August 22 (Zaporizhya) until August 29 (Odessa) and August 25 in Vinnitsa for 2011 and 2012. The second period of increased Ambrosia pollen concentrations occurs after September 5 each year but has been shifting to a later time being September 2, 2010 in Dnepropetrovsk and September 5 in Vinnitsa in 1999 and Zaporizhya in 2007 later occurring September 18, 2012 in Vinnitsa. Greater ragweed counts are now being seen later in Vinnitsa including 200 p.g./m 3 on September 18 versus 100 August 25, 2012). In 2012 the seasonal peak was observed for the second ten-day period of September in much of Ukraine, something not seen before. In 2010 and 2013 rapid increases of ragweed pollen were seen in the second ten-day period of August, two weeks earlier than usual being greater than the mean seasonal maximum on August 27, 2010 (102 p.g./m 3 versus 76 p.g./m 3 ) in Vinnitsa with 2013 ragweed pollen count increased to 82 p.g./m 3 on August 11 peaking at 92 p.g./m 3 on August 27. Seasonal maximum in Zaporizhya was on August 19, 2013. Conclusion: The changing pattern of ragweed pollination in Ukraine with early ragweed pollen count increases for the second ten-day period of August and changes in maximum in September may be evidence of the primary impact of temperature increase on Ambrosia season in Ukraine due to global warming. Background: Elevations in serum tryptase are used to support the diagnosis of anaphylaxis. Although tryptase has been studied in anaphylaxis to insect stings and drugs, it has not been measured systematically in patients who have systemic reactions (SRs) to immunotherapy (IT). Methods: This study was an IRB-approved, prospective cohort study of aeroallergen or venom IT patients who experienced SRs during the 30-minute post-IT period and were treated as clinically indicated. Consent and serum total tryptase were obtained after a onehour observation period when the patient was ready for discharge. A baseline tryptase was drawn 2-7 days post SR. Tryptase levels were determined using commercially available ELISA kits. Results: Twelve patients had 13 reactions over a 20-month study period. Patient and SR specifics are shown (Table 1 ). All reactions were grade 1 or 2 in severity. Flushing was the most common, objective symptom. One or 2 doses of epinephrine were administered for all SRs. All SRs were non-life-threatening and occurred during the build-up phase of immunotherapy. Median time from SR onset to resolution and from SR onset to tryptase lab draw was 26 and 85 minutes, respectively. No elevation in tryptase post-SR or change from reaction to baseline tryptase (>2 ng/ml) was observed. Discussion: Tryptase is a marker of mast cell degranulation with clinical utility, but it is not consistently elevated in all cases of anaphylaxis. In this study, SRs after IT were of short duration and mild severity. Unlike field anaphylaxis due to insect stings, post-IT SRs were promptly recognized and treated. The low total serum tryptase levels may reflect post-epinephrine effects of a terminated systemic reaction. Additionally, IT-associated SRs may represent a unique or undefined phenomenon involving complement, anaphylatoxins, kinins, or lipid mediators. Basophils rather than mast cells may be activated in the early stages of IT-associated SRs. These data suggest that tryptase is of lim-Introduction: The most common adverse events (AEs) with sublingual immunotherapy tablets (SLIT-T) are local allergic reactions of the mouth and throat. These AEs are expected as the allergen extract presumably elicits an IgE-associated mediator release from mast cells located in oral tissues. Our objective was to report the duration of local allergic reactions in response to SLIT-T for 3 different allergens to characterize the reaction patterns. Methods: Symptoms and duration in minutes of local allergic reactions after SLIT-T administration were collected during phase 1 studies of Timothy grass SLIT-T 2800 BAU (MK-7243) and short ragweed SLIT-T 12 Amb a 1-U (MK-3641), and during phase 1 and 2b studies for house dust mite (HDM) SLIT-T 12 DU (MK-8237; all Merck/ALK-Abelló). Additionally, the duration in days (number of days that an event recurs) was collected for local allergic reactions during the phase 3 trials for each of these tablets. IRB approval and informed consent was obtained from all subjects. Results: The median durations in minutes for the most common local adverse reactions after SLIT-T or placebo administration are shown in the Table. For each of the events, reactions typically resolve within minutes. Events generally recur over several days prior to resolution of the events. The median recurrence of symptoms after active treatment varies in duration, ear pruritus (4 to 14 days); oral pruritus (5 to 8 days); throat irritation (5 to 9 days). Conclusions: The occurrence and duration of local adverse reactions related to SLIT-T were similar across studies, and appear to be a class effect consistent with an IgE-mediated response. Although cautious interpretation is required due to the limited sample size, the data reveals that the reactions generally resolve within minutes of SLIT-T administration and are experienced for less than 2 weeks. SLIT-T *Sum of daily durations throughout entire 28-day trial. †Duration on day 1 of treatment. AE=adverse event; ND=not determined; SLIT-T=sublingual immunotherapy tablet. rospective survey was used to evaluate the association between the loss of skin test reactivity during IT and long-term clinical efficacy. Methods: Patients (Age 18-65) were mailed a follow-up survey if they had stopped IT prior to 2010 and met the following criteria: Skin test (+) to mountain cedar (MC) with ≥ moderate AR during MC pollinations. Achieved 1:1 vial dosing for > 12 months and had repeated skin testing prior to IT discontinuation. Results: Of 200 surveyed, 28 of 40 respondents met inclusion criteria. Among the 14 patients reaching the monthly goal of 0.5cc (mode volume), 100% reported "significant improvement" of AR while receiving IT and a fall in skin test grading by ≥ 2 grades was seen in 93%. A sustained complete remission (CR) of symptoms was reported by 43% while off IT for 5 to 10.1 years (median 6.9 yrs). In contrast, among those who had received a lower maintenance dose (Mean = 0.22 ± 0.17cc.), a fall in skin test reactivity was seen in only 42% and CR was reported in only 21% (n=14). No differences between groups were observed for: initial symptom severity or skin test grading, subjective treatment efficacy (while on IT), or the IT duration. Conclusion: "Real-world" JTF-compliant IT is highly effective, both during and after treatment courses. Aeroallergen IT, formulated and administered per JTF-recommendations, can induce allergen-specific skin test anergy in a dose-dependant manner. The induction of a persistent remission was significantly more common among those in whom the loss of skin test reactivity was observed. A. Gonzalez-Estrada * , L. Cuervo Pardo, B. Ghosh, F. Pazheri, M. Smith, K. Zell, X. Wang, D.M. Lang, Cleveland, OH. Background: Asthma affects over 300 million people globally, including 25 million in the US. Patients with asthma frequently use the internet as a source of information (Cabana MD, Le TT. J Allergy Clin Immunol. 2005). YouTube, a video sharing website, is one of the three most popular websites (http://www.alexa.com/topsites). We sought to determine the educational quality of asthma YouTube videos. Methods: We performed a YouTube search using the keyword "asthma" from June 4-8, 2014. The 200 most viewed relevant videos were included in the study. Asthma videos were analyzed for characteristics, source and content. Source was further classified as asthma healthcare provider, other health-care provider, patient, pharmaceutical company, and professional society/media. A scoring system was created to evaluate quality (-10 to +30 points). Negative points were assigned for misleading information. Five blinded reviewers scored each video independently; a mean score was calculated by video source. Two-tailed analysis was performed for video characteristics, ANOVAs were performed to compare scores by video type, and intraclass correlation was used to assess similarity in scoring by reviewers. Results: Two hundred videos were analyzed, with a median of 18,073.5 views, 31.5 likes, 2 dislikes, and lasted a median of 172 seconds. More video presenters were male (60.5%). The most common type of video source was other health-care providers (34.5%). The most common video content was alternative treatments (38.0%), including live-fish ingestion, reflexology, acupressure/acupuncture, ayurveda, yoga, raw food/vegan/gluten free diets, marijuana, Buteyko breathing, salt therapy, etc. Scores for videos supplied by asthma healthcare providers were statistically significantly different from all other sources (p < 0.001) and had the highest average score (9.91). Table 1 summarizes all scores by video source. Overall, there was a high degree of agreement among reviewers (rho = 0.847; p < 0.001). Conclusion: We found that YouTube videos on asthma are frequently viewed, but are a poor source of health care information. Videos by asthma health-care providers were rated highest in quality. The Allergy/Immunology community has a clear opportunity to enhance the value of educational materials via YouTube. Gavino, R. Rossen, F. Orson, A. Casillas, Houston, TX. Introduction: Local reactions are commonly noted during allergen immunotherapy (IT) administration. These are usually mild and self-limiting; with more than 80% of reactions smaller than the size of a palm (8-10 cm). Studies have shown that local reactions may not be predictive of subsequent systemic reactions. A common practice to empirically enhance safety is to repeat or reduce the IT dose based on the size of the post-administration wheal. Conservative management of local reactions less than 25 mm in size may lead to inadequate therapy. We report the incidence of local and systemic reactions with IT administration and the time taken to reach maintenance therapy at our institution which follows a conservative management of local reactions less than 25 mm in size. Methods: We reviewed data on patients receiving aeroallergen immunotherapy at an academic medical center where dosing practices are based on a protocol that is altered according to local wheal size after IT administration. Patients on immunotherapy for 23 weeks or more were included. Post injection reactions were graded from 1-5, and dose adjustments were made accordingly (see table) . Other factors which led to dosage changes were a change to a new vial and time delays over two weeks since last dose. Results: Fifty-six patients received 6633 IT injections over 0.5-7 years (mean of 2.1years ±1.2 years). Local reactions were observed in 89% of patients with an incidence rate of 13% (grade 2 = 37%, grade 3=15% & grade 4=48%). Half of the reactions (52%) were less than 25 mm. Thirty-eight percent of the reactions occurred during build up phase delaying the initiation of maintenance therapy. The mean time taken to reach maintenance (1:1 concentration) was 7.6 ± 3.4 months with 61% of all patients reaching this level over 5-22 months. Only 4 patients (7%) reached the final therapeutic IT dose. Conclusions: We note that more than half of the reactions were smaller than 25 mm in size. Most (93%) patients did not achieve the prescribed maintenance IT dosage over the time period studied. It may be possible to increase efficacy of IT by changing the dose adjustment criteria for reactions that are smaller than 25 mm in size. A. Crans Yoon *1 , W. Crawford 1 , J. Sheikh 1 , A. Gong 1 , R. Nakahiro 2 , M. Schatz 2 , 1. Los Angeles, CA; 2. San Diego, CA. Introduction: A new Healthcare Effectiveness Data and Information Set (HEDIS) asthma measure has been implemented, however it is unknown if meeting this care measure is associated with improved asthma outcomes. We examined if the HEDIS Medication Management for people with Asthma measure ("MMA measure") associates with asthma outcomes. Methods: Administrative data was used to identify 30,040 patients who met the HEDIS criteria for persistent asthma during 2012. They were classified as meeting or not meeting the MMA measure at the 75% and 50% adherence thresholds. Association between MMA adherence in 2012 and asthma outcomes in 2013 was assessed. Patients who were not MMA adherent but met the HEDIS asthma medication ratio measure were compared to those adherent to the MMA measure. Results: Patients who met the 75% or 50% MMA measure in 2012 had no difference in asthma related hospitalizations, ED visits, short-acting beta agonist (SABA) dispensing, and oral steroid dispensing events in 2013 compared to those who did not meet the measure. Stepwise comparison of patients who were 75% compliant, 50-75% compliant and < 50% compliant also showed no difference in these outcomes. To address confounding by patients who met the measure due to having a later index date, patients who met the 75% MMA measure with index dates late in the year were compared to those who did not meet the 75% measure whose index dates were early in the year and no difference was found in their 2013 asthma outcomes. Additionally, patients who met the 75% and 50% MMA measures were compared to those who did not but had an asthma medication ratio of >0.5. Asthma outcomes of hospitalization, ED visits and greater than 6 SABA canisters dispensed were higher in the 75% adherent group Introduction: In clinical practice where time and resources are limited, the asthma control test (ACT) and the childhood asthma control test (C-ACT) were developed by Nathan et al and Liu et al, respectively , to identify poorly controlled asthma. The ACT contains questions for the patient >12 yrs of age and the C-ACT contains questions for the caregiver and child 4-11 years of age. The goal of this study was to evaluate whether patients are on optimal asthma treatment based on subjective reporting of asthma control from the child and the parent as assessed by the ACT/C-ACT. Methods: A random selection of office visits made by children, ages 4-20 years, with asthma during 2013 was surveyed. We reviewed 171 charts: 114 children 4-11 years completed the C-ACT with their caregivers; 57 children 12-20 years completed the ACT. For children ages 4-11 years old, a comparison was made between the responses of children and their caregivers on their measure of asthma control. All surveyed charts were examined for step-up in medications if they had uncontrolled asthma (ACT/C-ACT score < 20) or appropriate maintenance or decrease in medications if controlled. Results: In children ages 4-11 years, it was noted that while caregivers' ACT scores had a moderate correlation (R 2 = 0.52) with their child's score, the caregiver's score was, on average, 7% higher than the child's score, suggesting that caregivers may perceive their child's asthma as more controlled than what the child reports. In children ages 4-11 years, 39% with uncontrolled asthma received an appropriate increase in medications, while 76% with controlled asthma were maintained on their medication or decreased. In children > 12 years of age, 36% with uncontrolled asthma received an increase in medications, while 81% with controlled asthma were maintained on their medication or decreased. Conclusion: Although results suggest a discrepancy between ACT scores for uncontrolled asthma and subsequent step-up in asthma management, there are other objective factors which play a role in determining asthma management, such as the pulmonary function test and physical exam. Analysis of these measures along with the ACT should be considered for asthma management. A communication gap about asthma symptom severity might exist between caregiver and child, which may be mitigated through patient education. P. Agarwal * , A. Chowdhary, S. Gaur, Delhi, India. Background: High attenuating mucus (HAM) impaction and other radiologic features (ORF) have been considered as important radiologic parameters while diagnosing and classifying patients of Allergic bronchopulmonary aspergillosis (ABPA). However, the exact significance of these radiologic findings is controversial. Method: A total of 63 patients diagnosed as ABPA based on the Rosenberg-Patterson criteria were categorized on the basis of HRCT findings into 4 groups; ABPA-S (Serological), ABPA-CB (Central bronchiectasis), ABPA-CB-HAM and ABPA-CB-ORF. These groups were studied for their baseline characteristics at initial visit and their clinical, serological and functional severity were assessed on follow up visits and ABPA exacerbations. IEC approval and informed consent was obtained from all research subjects. Results: The prevalence of HAM and ORF was found to be 9.5% and 41.3% respectively. A statistically significant higher correlation was observed for expectoration of brownish black mucus plugs (P < 0.03), anorexia (P<0.05), weight loss (P<0.008), mean number of ward admission per year (P<0.015), mean number of ABPA exacerbations per year (P<0.001), mean total IgE levels (P<0.001) in patients with HAM on the initial visit. Also, significantly more number of ORF patients had history of anti tubercular treatment for sputum positive tuberculosis (P<0.01). Furthermore, severe and very severe grades of obstruction on spirometry were observed in ORF group. The prospective analysis revealed mean serum total IgE levels (P<0.03) to be significantly high in HAM group, whereas, mean specific IgE levels against Aspergillus fumigatus were found to be significantly higher (P<0.007) in patients with central bronchiectasis . The follow up HRCT done during exacerbations detected presence of HAM in 3 patients of ABPA-CB, 1 of ABPA-CB-ORF and 4 of ABPA-CB-HAM. A high serum total IgE and specific IgE values were found in HAM impaction but were not statistically significant. Finally, comparisons made in the exacerbation group between HAM positive and HAM negative patients were also statistically insignificant. Conclusion: HAM and ORF appear to be associate findings in ABPA patients which may or may not influence the severity of the disease. Also, these findings could not be used as a diagnostic tool independently and do not predict the outcome. . Introduction: Medication non-adherence leads to increased pediatric emergency visits and hospitalizations. Tools to predict adherence in adults with asthma have been successfully validated. No tool currently exists in pediatrics that reliably predicts adherence to use of controller asthma medications. We developed a PAAT to quantitatively predict adherence in children with persistent asthma. Methods: Patients between ages of 2 and 18 with a new diag-nosis of persistent asthma who were started on inhaled corticosteroids were prospectively enrolled with informed consent. Each subject completed the PAAT. The PAAT was scored such that a higher score suggested better adherence. Refill history was assessed through phone calls to the subject's pharmacy at 1 week, 3 months, and 6 months after initial visit. Adherence was defined as 4 or more medication refills at 6 months. IRB approval and informed consent was obtained from all research subjects. Results: At 6 months, only 10/74 subjects (13.5%) of patients filled 4 or more controller inhalers. The median number of refills for controller medication at 6 months was higher for subjects who answered "agree or strongly agree" compared to "neutral" to the question regarding if their controller was necessary (median 3 vs. 2, p=0.028). There was no difference in total PAAT score between the adherent and non-adherent group (20 vs 18.5, p=0.406) . Additionally, there were no clinically significant differences in refill history for subjects for all other questions. Conclusions: We have demonstrated that the vast majority of patients receiving a new controller medication for their asthma are non-adherent. The PAAT was able to identify that adherence is better in patients who believe that their controller was necessary for their asthma. Asking this question during a pediatric visit may indicate a patient at higher risk for non-adherence, and may improve patient outcome through early intervention focusing on essential role of medication education. Unfortunately, total PAAT score failed to identify adherent versus non-adherent patients in our asthma patient population. Further investigation is necessary to determine the validity of the tool to predict a child's adherence to a controller medication. Graph, Results of exposure, allergen sensitivity and morbidity. (+) ssIgE was determined as ≥ .35 kU/L." Group 1 is made up of children with reported exposure to roach and positive roach SPT. Group 2 is made up of children with reported exposure to mouse and positive mouse SPT. Group 3 is made up of children with positive roach SPT but without reported exposure to roach. Group 4 is made up of children with positive mouse SPT but without reported exposure to mouse. Group 5 is made up of children with reported exposure to roach and positive roach ssIgE. Group 6 is made up of children with reported exposure to mouse and positive mouse ssIgE. Group 7 is made up of children with positive roach ssIgE but without reported exposure to roach. Group 8 is made up of children with positive mouse ssIgE but without reported exposure to mouse. Introduction: X-linked hyper-IgM syndrome (XHIM) is a primary immunodeficiency with absent IgG, IgA, IgE and normal/elevated IgM due to defects in the CD40 ligand (CD40L) gene. Hematopoietic stem cell transplantation (HSCT) is the only curative modality, but it carries significant risks, suggesting the need for improved methods of treatment. Previous studies using CD40L-/-bone marrow corrected by retroviral-vector transfer of CD40L cDNA in mouse models resulted in abnormal lymphoproliferation due to unregulated expression of the gene. Hypothesis: Custom TAL effector nucleases (TALENs) or Clustered Regularly Interspaced Short Palindromic Repeats (CRISPRs), combined with the effective delivery of a homologous donor sequence containing normal CD40L DNA, will allow for targeted integration and provide physiologic expression of the endogenous CD40L gene to safely provide longterm immune reconstitution. Methods/Data: TALENs targeting the 5' UTR of the CD40L gene were created and their function validated using a surveyor endonuclease assay. Allelic disruption of up to 31% at the target locus in K562 cells was achieved. In order to evaluate the capacity for targeted integration of a cassette at the cut site, K562 and Jurkat cells were electroporated with the TALEN pair and a donor molecule with a promoterless GFP reporter gene flanked by homology arms that parallel the cut site. In/Out PCR using a forward primer within the GFP region and a reverse primer in the genomic DNA outside the donor molecule demonstrated proper integration into K562 cells. Targeted insertion of the GFP reporter into the CD40L locus should also provide a measure of physiologic induction of CD40L expression. Thus, expression of the GFP reporter was evaluated in Jurkat cells (which naturally express 60% CD40L), with up to 10% detected by FACs and increasing to 22% upon PHA activation. In addition, CRISPRs targeting a patient-specific mutation in intron 3 achieved >50% gene disruption in K562 cells. Co-electroporation with a template donor modified to contain a unique restriction enzyme site demonstrated site-specific gene integration by enzyme digest and gel electrophoresis. Conclusion: These results demonstrate that site-specific modification at CD40L is achievable and that physiologic expression of the endogenous CD40L gene could provide a viable therapy for immune reconstitution in XHIM. R. Steele * , M. Littlefield, I. Voloshyna, M. Davis-Lorton, M. Aquino, L. Fonacier, A. Reiss, Mineola, NY. Introduction: Classically activated M1 macrophages drive Th1 responses, while alternatively activated M2 macrophages drive Th2 responses. A shift away from Th2 toward Th1 is associated with decreased incidence of atopy. Anti-IgE monoclonal antibody (mAb) therapy (omalizumab; a humanized IgG1 mAb) is an FDA approved treatment for allergic asthma and chronic urticaria. In recent studies, patients with non-allergic asthma and those with low serum IgE showed improvement in clinical measures with omalizumab treatment independent of IgE level. This may represent an unrecognized immunomodulatory effect of omalizumab. IgE-independent effects of this mAb in antigen presenting cells were examined in polarization of the THP-1 human monocytic cell line in an IgE free system. Methods: THP1 were differentiated into adherent macrophages (PMA), then stimulated with IFNγ/LPS and IL-4 to polarize them into the M1 and M2 state, respectively. Cells were stimulated with omalizumab at varying concentrations (0.1, 1, 10 mg/mL) ± IFNγ/LPS and IL-4 (during and after polarization). Then mRNA was isolated and subjected to QRT-PCR to examine profile markers for M1 (CCR7 and IL12), and M2 (CD163 and CCL17). Results were normalized to GAPDH. Results: Costimulation with mAb-IFNγ/LPS upregulated M1 markers CCR7 (17-fold) and IL12 (3-fold) above polarization with IFNγ/LPS alone. Treatment of already polarized cells with mAb did not significantly impact transcription of CCR7 or IL12. The M2 marker CD163 was downregulated by costimulation by mAb-IFNγ/LPS (15-fold) vs. IFNγ/LPS alone (8-fold) compared to PMA controls. IL-4 did not significantly affect M1 or M2 markers. M2 marker CCL17 transcription was enhanced by omalizumab alone (11-fold) and with IFNγ/LPS (9-fold) over PMA controls. Conclusions: This proof-of-concept study suggests that IgG mAB enhances transcription of M1 markers in THP1 cells and attenuates transcription of some M2 markers. This effect may be due to the Fab portion (omalizumab specific) or be isotype specific (IgG1). Interestingly, CCL17 is an inducer of chemotaxis in T-cells, particularly Th2 cells that play a critical role in atopic disease and this was upregulated by our mAb. The interaction of the mAb may represent a point of immunomodulation by omalizumab not previously recognized, or a novel immunomodulatory effect of IgG1. SNP [rs731236 A> G], ApaI [rs7975232 A> C], BsmI [rs1544410 C> T] and FokI [rs2228570 A> G] with susceptibility to asthma in children from the Caribbean Coast Colombian. Methods: This was a case-control study that included 1000 individuals : 500 asthmatic children belonging to a poor community and 500 non asthmatic children from the same socioeconomic characteristic living in Barranquilla a city on the north of the Caribbean coast of Colombia. The SNPs were genotyped by RT-PCR and TaqMan ® probes. Allelic and genotypic frequencies were estimated using a Arlequin v3.5 software; D'and r2 statistic Linkage disequilibrium (LD) were studied using Haploview v4.2 software and associated haplotypes were identified by haplo.stats v1.6.8 software package R v3.0.2. Results: Our results showed that only the SNP BsmI [rs1544410 C> T] was associated with the disease with a significantly higher frequency of the CC genotype (57%, n = 280) in the asthmatic groups compared with controls(49.2% n = 244)It was also found that the frecuency of CT genotype was 35.6% in cases, n = 175, compared with the controls (43.8%, n = 217), p = 0.031. Haplotype analysis showed that the haplotype A / C / T / G was found to be a protective factor for asthma (OR = 0.49, 95% CI = 0.26 to 0.88, P = 0.028), while haplotype A / C / C / G was showed as a risk factor for disease susceptibility (OR = 1.28, 95% CI = 1.03 -1.60, p = 0.049). The LD analysis showed that the best D'and r2 found in SNPs block understood by TaqI, ApaI and BsmI; was D '= 0.753 and r2 = 0.565. Conclusion: This is the first study conducted in asthmatic Colombian children searching for the association of the VDR polymorphisms with pediatric asthma. Our results demonstrate that the VDR gene locus is associated with susceptibility of asthma in this specific admixed ethnic group of children seated in the Colombian Caribbean Coast. J.M. Diaz *1 , X. Xue 2 , M. Solanki 2 , M. Gupta 2 , P. Chatterjee 2 , V.R. Bonagura 2 , C. Metz 2 , 1. North Bellmore, NY; 2. Manhasset, NY. Background: The obesity epidemic has contributed to the development of a new phenotype of "obese asthmatics" who are steroid resistant. To develop more effective treatments for these asthmatics, it is important to understand the etiology of this resistance. Using a mouse model of obese asthma, we previously reported a macrophage-(MΦ) predominant infiltrate in the lungs. We hypothesize this non-eosinophilic cell composition contributes to steroid resistance. Because MΦ can be pro-inflammatory (M1) & anti-inflammatory (M2), we examined the infiltrating MΦ in lean vs. obese asthmatic mice. Methods: Mice (C57BL/6, males) were fed a regular "lean" or high fat chow (60% fat). After 6 months, mice were given intranasal saline or house dust mite extract (HDM), 5 days/week for 4 weeks. During the last 2 weeks, mice were given either saline or dexamethasone (dex) (1 mg/kg, IP) 5 days/week. After pentobarbital euthanasia, bronchoalveolar lavage (BAL) and lungs were collected. Differentials were established using Modified Wright stain. Quantitative PCR (qPCR) using lung tissue was performed using F4/80 (MΦ marker), Fizz (M2), Arg1 (M2) & Nos2 (M1) primers. Results: The absolute cell count in the BAL was low in untreated obese & lean mice (<1x10 6 cells/mL), while HDM exposure significantly increased cell count in both (3x10 6 ). Dex significantly decreased BAL counts in lean-HDM mice (<1x10 6 ) but not in obese-HDM mice (2.5x10 6 ). Both HDM groups had a significant increase in eosinophils when compared to saline control mice. BAL of lean-HDM mice was comprised of equal numbers of MΦ & eosinophils, while obese-HDM mice had more MΦ & fewer eosinophils. Obese-HDM-dex mice had almost no eosinophils, but high levels of MΦ. qPCR revealed increased expression of F4/80, M1 (Nos2) & M2 (Fizz, Arg1) markers in obese-HDM vs. lean-HDM mice in comparison to their controls. Finally, DEX-significantly reduced M2 marker expression but did not reduce M1 expression in obese-HDM mice. Discussion: Using a novel model of obesity & asthma our findings suggest a potential role of dex-resistant M1 MΦ in the etiology of steroid resistance in the obese asthmatic. Because these findings are consistent with what has been observed in the obese population, this model might be useful to test new treatments that target reducing MΦ infiltration or shifting M1 pro-inflammatory to M2-antiiinflammatory MΦ. LONGITUDINAL STUDY OF CVID ILD. P.J. Maglione * , C. Cunningham-Rundles, New York, NY. Introduction: Evidence of interstitial lung disease (ILD) is a frequent radiologic finding in common variable immunodeficiency (CVID), however, the long-term consequence is unclear. We retrospectively examined CVID patients who had pulmonary function testing (PFT) over the duration of 20 months or more to gain insight into the natural history of CVID ILD. Methods: Retrospective chart review and statistical analysis was performed for 10 subjects with CVID who had CT chest evidence of ILD and PFT covering at least 20 months. CT evidence of ILD was defined as the presence of numerous pulmonary nodules, ground glass opacity, or interstitial infiltrate as noted by boardcertified radiologists in the medical record. Patients who received rituximab had PFT measured 6-12 months after the completion of a single course of treatment. Results: Of the 10 patients studied, 5 had evidence of progressive ILD indicated by (1) DLCO less than 50% of predicted, (2) DLCO less than 80% of predicted that decreased by 20% or more during the duration of the study, or (3) FVC that decreased by 20% or more during the duration of the study. Initial PFT parameters and conventional laboratory tests (quantitative immunoglobulins, CBC with differential, alkaline phosphatase) did not statistically differentiate these two groups, however initial serum IgM trended higher in patients with progressive ILD (68±28 mg/dL vs. 13±4, P = 0.08). Four of the 5 patients with progressive ILD received treatment with rituximab and had a significant improvement in FVC (73±1% vs. 62±2%, P = 0.01), but no significant restitution of other PFT parameters. Conclusions: Half of the CVID patients with CT evidence of ILD in this retrospective study had worsening of PFTs suggestive of progressive lung disease. Initial serum IgM trended higher in patients with progressive ILD, but other conventional laboratory parameters did not significantly differentiate those who went on to have worsening PFT. Treatment with a single course of rituximab in patients with progressive ILD resulted in improvement of FVC. Larger studies are indicated to define the natural history of CVID ILD and determine the optimal indication and mode of treatment 31 MULTIPLE UNRELATED HETEROZYGOUS GENE DEFECTS PRESENTING AS A PRIMARY IMMUNODEFICIENCY. P. Abghari * , E. Secord, P. Poowuttikul, Detroit, MI. Introduction: Many syndromes of primary immunodeficiencies (PID) are well described and have been identified by genetic analysis. We present a case of a boy with hypogammaglobulinemia, malabsorption and recurrent skin lesions with multiple heterozygous defects on genotyping; any of these alone would not likely be clinically significant, but together constitute an immune deficiency. Case Description: A 2 year old Chaldean boy was born full-term to consanguineous parents. At approximately 7 weeks of age he presented with skin lesions over the umbilicus and buttock. Biopsy revealed exuberant epithelial hyperplasia. By 6 months of age he developed chronic diarrhea, oral thrush, bacteremia, failure to thrive, poor wound healing and wound dehiscence. Laboratory evaluation at 6 months revealed hypogammaglobinemia, inadequate pneumococcal titers, normal oxidative burst, normal CD11, CD18, CD54 levels, normal T, B and NK cell populations and normal mitogen proliferation assays. Intravenous immunoglobulin (IVIG) infusions were initiated. The diarrhea and skin lesions responded to low dose prednisone, however despite the response, total parenteral nutrition was necessary for nutrition. Genetic testing revealed a negative panel for SCID, agammaglobulinemia, and chronic granulomatous disease. Whole exome sequencing revealed multiple mutations of unknown clinical significance. Two defects in DOCK 8 were detected, however protein was demonstrated to be present and functional. A heterozygous mutation for microvillus inclusion disease and a separate heterozygous defect for congenital malabsorption type 4 were identified as well. In addition, a heterozygous mutation of PRKDC, which has been linked to severe combined immunodeficiency when homozygous defects are present and a heterozygous mutation of the IKBKB gene which has also been linked to an immune deficiency when homozygous defects were identified. Finally, a heterozygous mutation of the IGFB2 gene, which codes for CD18 was identified. All were separately reported as of unknown significance. Conclusion: This case illustrates that multiple heterozygous defects, although not previously reported as related, together can present as a PID. Whole genome sequencing was able to provide us with results and should be considered more often in PID cases that are not straightforward and not easily diagnosed by immunology workup. PIDD patients are frequently managed by several physicians and care settings, and usually only see Immunologist 1-4 times per year. Given increased scrutiny on number of infections per year as a determinant of therapy and patient success, with risk that more frequent infections may contribute to more severe outcomes, we asked if present care model is sufficient for collecting rate of infection data and whether new data acquisition technology could improve data collection. We compared sample of 16 PIDD patients from 4 physicians in single academic immunology clinic by conducting chart review of office visit encounters, with sample of 40 PIDD patients from 23 physicians and 266 data collection points on CareExchange®, to compare time between data collections, frequency of reported infections, and infection rates per year. CareEx-change® is data collection software with multiple parameters focusing on the patient's infection rate, antibiotic use, provider visit, and overall health assessment. Chart review of PIDD patients on IG replacement from single academic center showed mean time between visits was 5.2 (1-24 months) and the mean number of infections/year was 1.2 (0-5). Mean age for this group was 21 (8-39 years). Primary diagnosis for single academic center was: 31.3% Bruton's, 43.8% CVID, 12.5% SCID, 6.3% Hyper-IgM. CareExchange patients had data collected on average 6.7 (1-15 times/year) and mean infection rate of 0.69 between infusions. Mean sum of infections from all sources at any one time between infusions was 1.38. Mean acute health care used between infusions was 0.4, and mean rate of new antibiotic usage between infusions was 0.25. Mean age for the CareExchange group was 39.2 years (range 5-75). Primary diagnoses for the group was: 48% CVID, 28% Hypogammaglobulinemia NOS 28%, 10% Congenital Hypogammaglobulinemia. This pilot study was limited, but revealed known gap in data collection, challenge of patient recall, and variability of physician questions/notes/visit schedule as compared to an automated system which can collect patient infection data as frequently as needed. There's no established acceptable rate of infections for a patient to be deemed well controlled. There is no well-validated system of acquiring data from home infusion patients. Further study is needed to validate utility of this automated uniform system of clinical data collection. S. Challa *1 , E. King 2 , K. Patel 2 , S. Anghel 2 , J. Brensilver 2 , L. Bielory 2 , 1. Springfield, NJ; 2. Summit, NJ. Rationale: PA generally leads to the use of broad-spectrum abx that may increase complications and cost. A PST pilot protocol was developed at Overlook Medical Center to help reduce the use of broad-spectrum/high-cost (BS/HC) abx and determine the cost effectiveness of PST. Methods: A retrospective analysis was conducted on patients who had PST performed by an allergist between November 2013 -May 2014. PST included prick and intradermal testing with major (Pre-Pen™) and minor (penicillin G) determinants followed by an oral amoxicillin challenge. Abx were de-escalated based on results of PST. Reduction in cost of abx, and adverse reactions were assessed. Cost analysis was based on the material cost of abx and skin testing components. Study was IRB approved. Results: A total of 38 adult patients had PST. Median age was 64 yrs; 97%(n=37) were allergic to penicillin alone; piperacillin/tazobactam (n=1), and a history of multiple abx allergies (n=9). Sixty three percent had a reaction >20yrs ago with cutaneous reaction being the most common reaction (45%). Aztreonam was the most common initial abx used prior to PST (55%).Urinary tract (26.3%) and intra-abdominal infections (18%) were the most common indications for abx treatment. Skin testing was negative in 38 (100%) patients but one patient had anaphylaxis to oral amox-icillin challenge following a negative skin test. A cost analysis was performed in 29 patients who had antibiotics switched to a beta-lactam after negative PST. Fourteen patients (48%) were switched to a narrow-spectrum agent (i.e. aminopenicillins, oxacillin, and non-pseudomonal cephalosporins). Total cost of therapy with a beta-lactam after PST was $3,518. Total cost of therapy if initial BS/HC abx prior to PST were continued was $14,670. Approximate cost for PST materials for 38 patients was $3,631. Overall cost savings was $7,521.The additional savings of impact on developing adverse reactions and superinfections (i.e. C. difficile) was not assessed. Conclusions: Following the implementation of a PST protocol, we observed a decrease in BS/HC antibiotic use in patients with previously documented PA. PST is a safe and costeffective procedure to serve as a negative predictor test for penicillin hypersensitivity mediated by IgE. K. Winkler * , T. Abramowitz Saadia, J. Moallem, New York, NY. Adverse effects of MMR vaccination, mostly self-limited and benign, are reported. Fever in up to 15% of vaccinees 6 to 12 days after vaccination(1, 2), transient rash in up to 5%, febrile seizure 5 to 12 days after immunization, as well as mild lymphadenopathy (2) are reported. Thrombocytopenia after MMR vaccination is reported in 1:25,000 to 1:2,000,000 (1). There is no report of thrombocytopenia resulting in severe hemorrhagic complications or death in immunocompetent recipients (2).Infrequency with which it occurs makes it a challenging diagnostic task for practitioners. 13 month old African American male presented to the emergency department due to two episodes of bloody vomitus.Two days prior he had fallen from a height of two stairs and sustained a rapidly enlarging hematoma on his forehead. A hematoma to the lip was noted on the day of presentation.10 days prior to presentation he was vaccinated with the MMR vaccine. There was no reported history of fever, recent upper respiratory illness or other illness.Physical examination in the ED demonstrated a fussy but alert child, HR 145, RR 36, T 100.2F, with a five centimeter hematoma on the forehead, hemorrhagic lesion on the upper lip, gingival bleeding and multiple petechiae on the upper and lower extremities.The rest of the physical examination was unremarkable.Laboratory investigation was significant for platelet count of 5K/uL, Hgb 8.4 g/dL and Hct of 24.7% on CBC. Indirect Coombs test and Stool Guaiac were positive.In PICU his hemoglobin further dropped to 4.0 g/dL, requiring transfusion. Bone marrow aspiration revealed absence of lymphoblasts with increased megakaryocytes and red blood cell precursors. He was treated with IVIG 1g/kg/day for 2 days and Methylprednisolone 4mg/kg/day for 4 days then continued on PO prednisone 2mg/kg/day to be continued for one week. CBC on the seventh day of admission showed platelets of 311 K/uL, and increase of hemoglobin hematocrit to 9.4 g/dL and 28%. ITP after MMR vaccination typically occurs within 6 weeks of vaccination (3).Mild bruising and petechiae are common (4). Hospitalization is rarely required.Transfusion is required in 1 of 1.8 million vaccinees (5).Platelet counts are typically >20,000 K/uL, higher than non-vaccine associated ITP (6).This is the first report of thrombocytopenia resulting in severe hemorrhagic complications in an immunocompetent recipient of the MMR vaccine. E. Willits * , A. Joshi, Rochester, MN. Introduction: Granulomatous interstitial lung disease (GL-ILD) develops in a subset of patients with common variable immunodeficiency (CVID) and is commonly confused with sarcoidosis. Little is known about the immunophenotyping of T and B lymphocyte subsets in GL-ILD. We reviewed the charts of four patients with CVID and GL-ILD. Methods: The presentation, laboratory and imaging studies, and treatment recommendations were reviewed. Each patient presented with thrombocytopenia and splenomegaly, and was subsequently found to have CVID and GL-ILD. Two patients, an 18 yr/M (#1), and a 12 yr/M (#2), presented with Evan's Syndrome. Results: All patients demonstrated significant hypogammaglobulinemia. Chest CTs were obtained in all patients and demonstrated diffuse nodular opacities. All patients underwent BAL and bronchoscopy to rule out an acute or chronic infection in addition to immunostaining and pathology studies on a lung biopsy sample. Tissue sam-ples were obtained to confirm the diagnosis of GL-ILD in three out of four patients. Finally, B-Cell Immunophenotyping was obtained for all patients and showed decrease in total memory B-Cells (CD27+),especially low class switched memory B Cells (CD27+M-D-). Conclusions: These four cases illustrate the association between low class switched memory B Cells with GL-ILD in CVID patients. Specifically, it was found that all patients had decreased percent (3/4) or decreased absolute number (4/4) of class switched memory B Cells. B cell immunophenotyping may therefore be a useful tool for identifying patients with CVID and granulomatous disease. Implications for early identification include early initiation of specific treatment regimens, since this subset of patients may have a poorer prognosis and decreased survival when compared to their granuloma-free CVID counterparts. In this specific population of CVID patients, the combination of Rituximab and Azathioprine has been shown in preliminary studies to result in improvement in pulmonary function tests and lung disease. The combination of low serum IgA levels, splenomegaly and low class switched memory B cells can be used to create a prediction model for the diagnosis of GL-ILD in CVID patients in future. Background: Rituximab is a chimeric anti-CD20 antibody used in the treatment of non-Hodgkin lymphomas, chronic lymphocytic leukemia, rheumatoid arthritis, granulomatosis with polyangiitis (GPA), and microscopic polyangiitis. Rituximab is comprised of human IgG1(κ) heavy and light chain constant regions with murine anti-human CD20 IgG1κ variable regions. Treatment with rituximab results in complement-dependent cytotoxicity, antibodydependent cellular toxicity and direct cytotoxicity to CD20-expressing B-lymphocytes. Pharmacokinetic studies suggest that serum levels of rituximab can still be detected 3 to 6 months after treatment with a half-life of elimination ranging from 5 to 78 days. As an IgG antibody, rituximab may cross the placenta and be secreted into human breast milk. However, it remains unknown what concentrations rituximab achieves in neonates in utero, and if breast feeding after rituximab treatment is safe. Methods: Rituximab was quantified by mass spectroscopy (MS) from serum of mother and infant. Results: A 31 year old primigravid woman with GPA required a fourth cycle of rituximab (375 mg/m 2 ) weekly for four weeks starting at 30 weeks gestation for management of headache stemming from pituitary involvement by GPA. She delivered a healthy term infant by cesarean section at 40 weeks gestation. The baby was exclusively breast fed without major health issues. Cord blood samples were not taken at the time of delivery. At four months post-partum, serum samples from the infant and mother were checked and shown in Table 1 . Trace amounts of peptides corresponding to both the heavy chain and light chain of rituximab were detected in the infant's and mother's serum by MS. Conclusions: Rituximab peptides were detected in the serum of an infant who was exposed to rituximab in utero and exclusively breast fed. The infant's B-cell population and immunoglobulin levels did not appear to be affected at four months of age (approximately 6 months after initial rituximab exposure). Measuring rituximab concentrations in breast milk may help to clarify if rituximab is secreted into breast milk. Laboratory results from infant and mother at 4 months post-partum Background: X-linked agammaglobulinemia (XLA) is a primary immunodeficiency characterized by low immunoglobulins and an absence of B-lymphocytes. XLA is secondary to a mutation of the Bruton tyrosine kinase (BTK) gene, which prevents the maturation of B cell lineages. Patients with XLA most commonly present with recurrent sinopulmonary infections and are at risk for severe enteroviral infections. We report the case of a 3 year old patient with a history of recurrent infections, treated for atypical Guillain-Barré syndrome (GBS), who was later discovered to have XLA. Case Report: The patient initially presented to our facility with proximal upper and lower extremity weakness, which progressed to near complete flaccid paralysis. After initial laboratory and radiographic studies were unremarkable, he was diagnosed with an axonal variant of Guillain-Barré syndrome. Empiric treatment with intravenous immunoglobulin (IVIG) was initiated. Despite therapy, his weakness progressed and the patient underwent plasmapheresis. After plasmapheresis he continued to receive pulse doses of IVIG for residual neurologic deficits. Prior to his diagnosis of GBS our patient had recurrent otitis media. In the following 24 months, he experienced recurrent infections such as sinusitis, pneumonia and an episode of S. pneumoniae bacteremia. Infections often occurred 4-6 months after receiving IVIG. He was thereafter found to have low total immunoglobulin levels. Flow cytometry revealed a total absence of CD19 B lymphocytes and nonprotective antibody responses to vaccination. Mutation analysis detected a c.595A>T nonsense mutation of the BTK gene, confirming the diagnosis of XLA. He was begun on monthly IVIG replacement therapy and transitioned to weekly subcutaneous immunoglobulin replacement. He has remained infection free since beginning a regular replacement program. Discussion: Patients with X-linked agammaglobulinemia often present with recurrent sinopulmonary infections. However, this patient presented with atypical GBS that required plasmapheresis and IVIG. Pulse IVIG therapy inadvertently resulted in intervals without recurrent infection that likely contributed to a delay in his diagnosis of XLA. This case demonstrates the importance of considering primary immunodeficiency in pediatric patients with a history of recurrent or severe infections and atypical neurologic syndromes. C.J. Calais * , Rockville, MD. Introduction: The purpose of this case is to shed more light on the link of alcohol-induce respiratory symptoms with aspirin exacerbated respiratory disease(AERD). Rationale: There is a distinct link between alcohol-induced respiratory symptoms in patients with AERD. A recent questionnaire based study of 50 patients which consisted of (4) clinical types (aspirin challenge confirmed AERD, aspirin tolerant asthma, aspirin tolerance with chronic rhinosinusitis, and a healthy control), characterized the prevalence of alcohol-induced reactions to be highest in patients with AERD. We present a case of a patient with confirmed AERD/Samter's triad and alcohol-induced respiratory reaction that experienced resolution of alcohol-induced symptoms with aspirin (ASA) desensitization therapy. Methods: Review of clinical course, documentation as well as ASA desensitization/challenge was conducted at Walter Reed National Military Medical Center Allergy Clinic. Results: Our 56 year old patient had a history of facial flushing and profuse rhinorrhea with ingestion of alcoholic beverages, which arose concurrently with his diagnosis of severe recurrent nasal polyposis, asthma, allergic rhinitis, anosmia, and ageusia. He first experienced hypersensitivity to motrin in 2008 followed by ASA hypersensitivity in 2012 for which he related the symptoms of cephalic flushing, profuse rhinorrhea and ocular watering all of which mirrored his symptoms with alcohol ingestion. ASA challenge/desensitization in 2014 elicited upper respiratory symptoms and a reversible decline in FEV1 of >10% from baseline. Since the desensitization therapy, he has remained on ASA 650mg twice daily and has regained his sense of smell, taste, and can now tolerate alcohol consumption. Conclusions: The pathogenesis of alcohol-induced respiratory symptoms association with AERD is unknown; however, current thought postulates that alcohol may have an inhibitory effect on the catabolism of leukotriene E4. This case lends clinical evidence that bolsters the idea of a common pathway for both conditions since ASA desensitization improved both disease processes. Clinically, allergists should be cognizant of this link and the possibility that such patients may be able to tolerate alcohol following successful aspirin desentization . A SUCCESSFUL TREATMENT OF SOLAR URTICARIA WITH OMALIZUMAB. M. Chong * , M. Aquino, M. Davis-Lorton, L. Fonacier, Mineola, NY. Background: Solar urticaria is a rare physical urticaria caused by ultraviolet radiation. Managing solar urticaria can be challenging as treatment options are limited to anti-histamines, sunscreen and UV light-hardening therapy, which are usually ineffective. As Omalizumab has been shown to effectively treat refractory chronic urticaria, we hypothesized that Omalizumab may be effective in the treatment of solar urticaria as well. We present a case of solar urticaria treated successfully with Omalizumab. Case Report: A 29 year old male was evaluated for a 9 year history of solar urticaria involving both exposed and lightly covered skin areas. He noted improvement in his symptoms while working as a construction worker in Florida for 4 years. However, upon return to New York 1 year ago he began to experience urticarial lesions every time he was exposed to sunlight despite the use of high-dose antihistamines (Zyrtec 40mg or Allegra 360 mg daily) and sunscreen. His urticaria was not exacerbated by other physical factors. Challenge tests were negative to visible and laser light. However, multiple erythematous macules developed within 15 minutes of sun exposure through a window pane and 5 minutes of direct sun exposure on a different site. A skin biopsy of the macular lesion revealed a superficial to deep perivascuar inflammatory cell infiltrate consisting of lymphocytes, histiocytes, neutrophils and eosinophils. The biopsy and clinical findings were consistent with a diagnosis of solar urticaria. The patient was placed on a monthly trial of Omalizumab 300 mg SQ for a total of 3 doses. With daily challenges of sun exposure, the frequency of urticarial lesions significantly decreased 7 days after the first dose with complete control of urticaria after an additional two doses of Omalizumab. Discussion: Omalizumab is a monoclonal anti-IgE antibody that is FDA-approved for allergic asthma and chronic urticaria. A few case reports have shown variable effectiveness of Omalizumab in the treatment of solar urticaria; one case showed resolution, one showed improvement and the last case did not show any effect on solar urticaria. To our knowledge, this is the first case reporting rapid symptom improvement within 7 days of initiating therapy. Although the evidence is limited, Omalizumab may be considered in patients with solar urticaria that is refractory to standard therapies with anti-histamines and sunscreen. Introduction: The term Immunoglobulin G4-related disease (IgG4-RD) refers to a newly recognized collection of fibroinflammatory disorders characterized by tissue infiltration of IgG4-positive plasma cells and eosinophils, obliterative phlebitis and elevated serum IgG4. While the clinical presentation of IgG4-related disease will depend on the affected organ(s), the histopathological features, which are the cornerstone of the diagnosis, are strikingly similar. Little is known about the pathogenesis of IgG4-RD, but TH2 cell responses are activated at the affected sites and patients may present with concomitant atopic symptoms. Case Presentation: A 66-year old Vietnamese man with a history of allergic rhinitis and chronic urticaria presented with a one year history of worsening neck lymphadenopathy and bilateral parotid gland swelling. Laboratory evaluation showed serum eosinophils 18.2%, IgE 270 mg/ml, total IgG 2870 mg/L, IgG1 640mg/dL, IgG2 1240mg/dL, IgG3 245mg/dL, IgG4 >300mg/dL, ESR 37mm/hr. Serum protein electrophoresis resulted in a total protein 8.1gdL, albumin 52.7%, alpha 2 globulins 7.5%, beta globulins 6.3%, gamma globulins 31.7%, with a possible monoclonal band present in the gamma region. Histopathological analysis of lymph node biopsy found plasma cell dyscrasia arising in the background of IgG4-related sclerosing sialadenitis. Immunohistochemical studies detected sheets of monotypic plasma cells positive for IgG4 (IgG4 infiltrate >150/hpf), IgG (IgG4/IgG ratio 80-90%), CD138, and Kappa light chain restriction, with periductal and perivascular onion-skinning fibrosis. The lymphadenopathy and parotid swelling improved following oral corticosteroid treatment. Discussion: Because of the intrinsic TH2 bias that characterizes IgG4-related disease, patients' initial clinical features may suggest an allergic condition. The presence of lymphadenopathy, swollen parotid or salivary glands or evidence of organ dysfunction should raise suspicion for IgG4-RD. Prompt recognition is key because, if untreated, the expanding inflammatory lesions can potentially destroy the involved organ. Moreover, up to 16% of patients develop malignancies during the course of the disease. Rationale: Eosinophilic esophagitis (EoE) is a clinicopathological Th2driven atopic disorder with an escalating prevalence. The condition is characterized by esophageal mucosal eosinophilic inflammation. Untreated EoE can lead to irreversible damage of the esophagus. Random food elimination appears to be effective; however, this regimen is empiric and is not evidence-based. The goal of the present study was to investigate the association of EoE and food allergy by means of an allergy evaluation, in order to determine the potential pathogenic role of specific food allergen(s), and to avoid prolonged random, unnecessary, and unsubstantiated empiric food elimination diet(s). Method: We evaluated 54 patients (n=54; mean age= 43 years) with the diagnosis of EoE based on clinical history of dysphagia, esophagogastroduodenoscopies (Figure 1) , biopsies with > 15 eosinophils/high-power field. Patients underwent food allergy evaluation by means of clinical history, allergy skin testing, and in vitro specific IgE ImmunoCAP testing. Study participants eliminated the putative offending specific food allergen for 16 weeks. Clinical and pathological improvement was assessed by a patient symptom scoring system (PSSS) and by upper GI endoscopy with esophageal biopsy to monitor treatment response. Results: The primary symptoms of EoE in our study included dysphagia (94%), food impaction (35%), and heartburn (96%). The common food allergens detected included wheat (58%), dairy (53%), egg (35%), soy (28%), peanut (25%) and tree nuts (21%). Avoidance of the above offending food allergens for a 16-week period resulted in improvement of symptoms, with a decrease (PSSS) of 98%, 100% and 90% in dysphagia, food impaction, and heartburn, respectively. Mean average density esophageal eosinophilic infiltration (eos/hpf) from mid-esophageal biopsies before and after specific food elimination diet decreased from 36 to 6 eos/hpf. Amelioration and sustained remission occurred in 82% of patients. Conclusion: The results of this study support the role of food allergy in EoE and illustrate the need for a collaborative gastroenterologic/allergic approach not only for successful management of EoE, but also for avoidance of unnecessary and potentially harmful, unsubstantiated exclusion diets. This requires a multidisciplinary collaboration of gastroenterologists, allergist-immunologists, pathologists, and nutritionists. A.L. Humphrey * , M. Reddy, J. Shroba, C. Ciaccio, Kansas City, MO. Introduction: The prevalence of food allergy has been difficult to assess, but previous studies report a range of 3.4%-8% in the pediatric population. It is unclear how or if geographic factors affect the prevalence of this disease. As our institution is the primary provider of pediatric allergy/immunology consultation within the urban core of Kansas City, we aimed to estimate the prevalence of food allergy in children residing in this region. Methods: This study was approved by the local Institutional Review Board. Pediatric outpatient clinic data from 2004-2014 was obtained through our hospital's electronic medical record. Patients with ICD-9 codes 995.3, 995.60-995.69, 995.7, 693.1, 708, V15.01-V15.05, and V15.09 were selected. United States Census data was used to estimate total population by ZIP code. Multiple encounters by the same patient were consolidated. Results: Representing 545 unique ZIP codes, 12,612 distinct food allergic children were seen from 2004-2014. Two thousand six hundred and fifty nine children were identified within the city's urban core (26 ZIP codes). The average prevalence of food allergy in this urban core was 5.2% with the range being 1.6%-10.3%. Conclusion: The prevalence of pediatric food allergy in urban Kansas City over the past decade is consistent with previously reported values. Further investigation is needed to determine if variations exist between urban, suburban, and rural settings. A. Tripathi *1 , L.J. Workman 1 , S.P. Commins 1 , R. Hamilton 2 , E.A. Erwin 3 , T.A. Platts-Mills 1 , 1. Charlottesville, VA; 2. Baltimore, MD; 3. Columbus, OH. Background: In the investigation of food allergy syndromes, serum assays detecting IgE antibodies (Ab) to food allergens use whole extracts on the solid phase, which contain specific allergen components in varying amounts. The interpretation of results assumes that the causative allergen components are adequately represented in the extract and that the presence and level of specific IgE titer to the whole extract of the relevant food(s) are diagnostic of the syndrome. IgE assays for beef or pork can underestimate the IgE Ab to a minor component of mammalian meat, αgal. The inciting food allergens in Eosinophilic Esophagitis (EoE) remain unclear, although IgE Ab to milk, wheat, soy, and peanut are frequently present in low titer. Method: IgE Ab to relevant allergens and their components were measured in the sera of adults and chil-dren with peanut allergy, delayed anaphylaxis to mammalian meat, or esophageal biopsy-diagnosed EoE. Assay methods included: ImmunoCAP (CAP) using whole and component extracts, CAP assays on serial dilutions (1:2 to 1:8) of sera, and ImmunoCAP ISAC (biochip assay for 112 purified allergens). Results:Analysis with ISAC correlated well with results using component-specific CAP assays for both peanut allergens (peanut allergy) and inhalant allergens (EoE). By contrast, no positive results for food allergens were found by ISAC in EoE sera that were positive for milk, wheat, or soy by CAP. Dilution assays showed no change (undiluted value vs. calculated titer) for peanut in peanut allergy sera or inhalants (dust mite and cat) in EoE sera in contrast, calculated titers up to six times the undiluted value were noted for foods in EoE sera (milk, wheat, and peanut) and in mammalian meat allergy sera (beef and pork). CAP assays for 5 milk components revealed positivity to minor components in over 50% of EoE sera. CAP assays for αgal revealed positivity in 100% of patients presenting with delayed anaphylaxis to mammalian meat. Conclusion: Differences in the results of the dilution assays demonstrate that assaying undiluted sera can significantly underestimate IgE Ab levels if the IgE Ab are directed against a quantitatively minor component of the extract on the solid phase. These results strongly suggest that the IgE Ab to milk and other foods in EoE sera are directed against a minor component that has not yet been identified. R. Gupta *1 , L. DeSantiago-Cardenas 2 , V. Rivkina 1 , 1. Chicago, IL; 2. Phoenix, AZ. According to the CDC, food allergy prevalence has increased by 50% between 1997 and 2011. Currently, approximately 1 out of every 13 children, or 2 children per classroom, has a food allergy. In addition, it is estimated that more than 15% of school aged children with food allergies have had a reaction in school and that 20-25% of epinephrine administrations in schools involve individuals whose allergy was unknown at the time of the reaction. For this reason, it is important for schools to have undesignated emergency epinephrine available for use in the event of an anaphylactic reaction. Purpose: The aim of this presentation is to demonstrate how Chicago Public Schools (CPS) was the first large urban school district in the United States to develop and implement a comprehensive stock epinephrine policy in accordance with state guidelines and present Year 1 findings. Significance: Understanding how CPS developed and implemented their Emergency Epinephrine Initiative will help districts across the United States appreciate the benefits and challenges of such an undertaking. Methodology: After updating a key health-related policy to include District Issued Emergency Epinephrine, EpiPens were distributed to all schools in the district prior to the beginning of the 2012/2013 school year. Data was collected on District Issued EpiPen usage based on school nurse progress notes and principal reporting mechanisms. Findings: During Year 1 of the CPS District Issued Emergency Epinephrine Initiative, 38 lives were saved because of the availability of stock epinephrine in schools across Chicagoland. Of these 38 individuals, 2 were school staff, and 36 were students between the ages of 3 and 19, with more than half of the reactions being first time incidents. Conclusion: After evaluating the distribution and tracking the usage of District Issued EpiPens, the CPS Office of Student Health and Wellness has learned key lessons which will inform the strategy for future years of the District Issued Emergency Epinephrine Initiative. As the first large urban school district to implement such a project, CPS is poised to be a national model of what a school district can do to save the lives of its students. respectively. The quality of exposure assessment ranged from measured exposure by validated questionnaire (n=2) to exposure surrogates, including plant (30 exposures), job title linked to job exposure matrices (n=3), geographic information system (n=2) to self-reported exposures without validation (n=16). Concerning outcome definition, no studies used the current clinical EPOS CRS definition (symptoms plus objective evidence of inflammation). Studies were thus classified based on the likelihood that their CRS definition represented true CRS: "probable" n = 17 for objective criteria; "possible" n = 8 for validated definition without objective evidence; and "least likely" n = 23 for varying definitions. Associations were assessed for the "probable" or "possible" studies. Fishing, grain farming, diving, cotton, pickling and glass blowing, World Trade Center rescue work (n=1 study for each), self-reported work exposure (n=5), second hand smoking (n=5), wood stove use (n=1) and air pollution (n=2) were the exposures that showed a positive association with CRS. Since most exposures were only evaluated by one study, no between-study evidence could be assessed. Among exposures with 3 or more studies, unclear or non-quantitative exposure assessment, biased study design, varied outcomes (eg. second hand smoking n=1 incident CRS, n=3 difficult-to-treat CRS with 3 different outcomes), and approach to analysis did not allow rigorous comparison or meta-analysis Conclusions: The published literature offers little evidence of whether and how occupational and environmental exposures may be associated with CRS. Studies are needed using current clinical case definitions and rigorous approaches to exposure assessment. K. Kumar * , A. Shah, Delhi, India. Introduction: Allergic rhinitis (AR), chronic rhinosinusitis (CRS) and nasal polyposis (NP) are known to coexist frequently. AR and CRS impair the quality of life (QoL) and also adversely affect the quality of sleep. NP remains largely undiagnosed and is thought to independently affect negatively the quality of sleep in patients with AR. Methods: The study comprised 106 consecutive patients with AR (males/females, 60/46), between 18 to 60 years, enrolled from outpatients department of VP Chest Institute, University of Delhi. IRB approval and informed consent was obtained from all research subjects. AR was diagnosed according to "Allergic Rhinitis and its Impact on Asthma" guidelines. CT-PNS, done in all patients, assessed the presence of CRS/NP. The patients were divided into three groups: AR alone (group 1), AR with CRS without NP (group 2) and AR with CRS and NP (group 3). To assess the severity of the disease and impact on QoL, patients responded to Visual Analogue Scale (VAS) and Sinonasal Outcome Test 22 (SNOT-22) respectively. To study the effect of NP on sleep, patients responded to Nocturnal Rhinoconjunctivitis Quality of Life Questionnaire (NRQLQ), the Epworth Sleepiness Scale (ESS) and the Pittsburgh Sleep Quality Index (PSQI). Results: The 106 patients were categorised as follows: group 1 with 34 (32%), group 2 with 36 (34%) and group 3 with 36 (34%) patients. Mean global VAS score increased from 7.12 in group 1 to 7.66 in group 2 and 8.03 in group 3 (P=0.004). Mean SNOT-22 score increased from 45.47 in group 1 to 66.83 in group 2 and 70.22 in group 3 (P=0.001). Similarly, mean NRQLQ score increased from 32.94 in group 1 to 49 in group 2 and 63.19 in group 3 (P=0.002). Mean ESS score too increased from 9.76 in group 1 to 11.14 in group 2 and 12.24 in group 3 (P=0.001). Mean global PSQI score also increased from 7.17 in group 1 to 9.67 in group 2 and 13.58 in group 3 (P=0.001). Conclusions: NP occurs in half of the patients with AR and associated CRS. The presence of NP significantly increased the severity of the disease and negatively impacted the QoL. Instruments to assess the effect on sleep highlighted the significant adverse impact of NP on the quality of sleep. Its presence denotes a more severe form of the disease, thus emphasising the need for early diagnosis. M. Duse * , A. Zicari, M. Nebbioso, F. Occasi, L. Leonardi, A. Zicari, Rome, Italy. Vernal keratoconjunctivitis (VKC) is a chronic disease affecting conjunctiva even though the immunopathogenetic mechanisms underlying this inflammation are unclear. Although many clinical and laboratory findings support the presence of an IgE-dependent immediate hypersensitivity reaction in VKC [2], the conflicting results concerning the association between VKC and atopy sug-gest that an IgE-independent mechanisms could also be involved. High-mobility group box protein 1 (HMGB1) has been recently identified as an important proinflammatory mediator with many characteristics similar to classic proinflammatory cytokines The aim of our study is to investigate serum levels of HMGB1 and circulating sRAGE in children affected by VKC before and after treatment with cyclosporine A (CsA) eye drops and in a group of otherways healthy children. Methods Twenty-four children affected by VKC aged between 5 and 12 yrs of life were enrolled. Twenty-four healthy children without atopy, ocular, and systemic disease, cross-matched for sex and age to patients affected by VKC, represented the controls. All children affected by VKC were treated with CsA 1% eye drops for 4 wks, and blood samples were collected before and 2 wks after the end of treatment while the controls underwent to a single blood sample at the time of enrollment. Statistical analyses were performed using SPSS (Statistical Package of Social Sciences, Chicago, IL, USA) software version 19. Ethical Committee approuved the study. Results Serum basal levels of HMGB1 and sRAGE were higher in children with VKC when compared with controls while, in patients affected by VKC, no difference was detected between atopic and non-atopic, and between ANA-positive and ANAnegative children. A significant reduction in serum HMGB1 and sRAGE levels was found after the therapy while CsA serum levels were negative. Discussion Our study gives a support to the definition of VKC as a systemic inflammation in which HMGB1 and its soluble receptors could play a role. We did not find any detectable levels of systemic CsA at the end of our study, but serum levels of HMGB1 and sRAGE showed a significant reduction after 4 wks of therapy. Our findings lead us to hypothesize that the topical administration of CsA may influence not only the ocular immunological responses but also the regulation of systemic inflammation. Background: Serum periostin (SP) has been proposed as a potential biomarker of TH2-related airway inflammation in asthma. However, allergic rhinitis (AR), another TH2 disease, commonly co-exists with asthma. There is limited information on periostin expression in rhinitis and whether this could confound the use of SP as a biomarker in allergic asthma. Here, we aimed to 1) To compare the presence and extent of periostin immuno-expression in nasal biopsies from subjects with both IN and OUT-OF-season (grass pollen) AR, perennial (house dust mite, HDM) AR, healthy subjects and in subjects with non-atopic rhinitis; 2) To assess whether airborne ragweed challenge affects SP levels in OUT-OF-Season ragweed-allergic subjects. Methods: Periostin immunostaining was assessed in nasal biopsies obtained from subjects with IN-season AR (n=17), perennial AR (n=13), non-atopic rhinitis (NAR, n=9), OUT-OF-Season subjects 6hrs after a nasal allergen challenge (NAC) (n=9) and healthy (n=9) and non-rhinitic subjects (n=9) (Figure 1 ). Serum SP was evaluated in subjects with ragweed-induced seasonal AR (n=15) and healthy (non-rhinitic, HNV) controls (n=5) before and after a 4 hour exposure to ragweed allergen in an environmental exposure chamber (EEC) on 2 consecutive days. Results: The extent of nasal biopsy periostin immuno-expression was significantly increased IN-Season AR (p<0.05), OUT-OF-Season NAC AR (P<0.05) and perennial AR (p<0.05) compared to non-allergic rhinitis and healthy subjects. No significant differences were noted between seasonal and perennial AR groups (p=0.950) or between healthy and non-atopic rhinitis groups (p=0.233) (Figure 1 ). There were no significant differences seen in SP after ragweed challenge compared to HNV studied in the EEC. Conclusion: Mucosal but not SP expression is increased in both seasonal and perennial AR compared to non-atopic rhinitis and HNV controls. This suggests SP does not reflect local nasal inflammation and is therefore not a reliable systemic biomarker for AR. Concomitant nasal disease should thus not interfere with the value of SP as a biomarker in asthma. Rationale: Studies have suggested that vitamin D deficiency is associated with increased prevalence of atopic disorders such as asthma, food allergy, and atopic dermatitis; however it has never been assessed in eosinophilic esophagitis (EoE). Our objective was to determine the levels of 25-hydroxyvitamin D in a cohort of patients with EoE. Methods: We measured total serum 25-hydroxyvitamin D using liquid chromatography with tandem mass spectrometry in adults (n=35) and children (n=34) with EoE. We compared the results to our patient demographics, EoE specific disease parameters, and markers of allergy using multiple logistic regression. Results: The median vitamin D level was 29 ng/ml. Patients with insufficient vitamin D (<30 ng/ml) were older (age 25.5 compared with 16.2 years) and had a higher body mass index (BMI) (median 25.2 compared with 19.8, p<0.001). Peak esophageal eosinophil counts were not different in vitamin D insufficient and sufficient patients; however, higher vitamin D levels were associated with higher tissue eosinophil counts (R=0.26, p<0.05). While there were no differences in total IgE or titers of specific IgE in insufficient and sufficient patients, positive skin prick test to peanut was more common in patients who were vitamin D insufficient with odds ratio (O.R.) 5.98 (95% CI 1.51-23.7). This result remained significant in a logistic regression model in which the results were adjusted for age and BMI (O.R. 7.57 (95% CI 1.66-34.6). Conclusions: In our group of adult and pediatric patients with EoE, vitamin D levels were low overall (median <30 ng/ml) indicating that this is a population at risk for vitamin D insufficiency. Interestingly lower vitamin D levels were not associated with higher esophageal eosinophil counts. The only marker of allergy associated with having an insufficient level in EoE patients was positive skin prick test to peanut. Poor understanding of allergic conditions can influence patient care through unnecessary diagnostic testing and/or management thus negatively impacting quality of care, cost, and outcomes. We conducted an anonymous electronic survey of pediatric (Ped) and internal medicine (IM) residents and faculty physicians at two tertiary care academic medical centers. Participants completed a 6 (IM) or 9 (Ped) item questionnaire regarding allergy evidence and/or guidelines. 409 surveys were collected (Ped faculty=129, Ped residents=106, IM fac-ulty=78, IM residents=96). An elective rotation in allergy (A/I) was completed by <50% of faculty and <20% of residents. When asked for the best first treatment for a patient experiencing urticaria and emesis immediately following ingestion of a known food allergen, physicians reported: epinephrine=64%, 1 st generation antihistamine=31%, H2 antagonist=3%, prednisone=1%. Only 50% of all IM physicians answered epinephrine (p<0.0001). Regarding allergies that should be inquired about prior to ordering an imaging study with radiocontrast media: iodine=80%, shellfish=40%, artificial dye=21%. IM physicians reported iodine (89 vs 73%) and shellfish (47 vs 34%) more than Ped physicians (p<0.05). Regarding contraindicated (CI) immunizations in egg allergic patients, they answered: influenza=85%, yellow fever=49%, measles, mumps, rubella (MMR)=18%, varicella=8%, rabies=4%. Ped physicians answered correctly regarding influenza (not CI) and yellow fever (CI) (49,50%) more than IM physicians (12,16%)(p<0.05). IM residents (22%) answered MMR more than any other group (p<0.05). Only 27% of all Ped physicians correctly identified common causes of IgE mediated food allergy in children <4 years old: egg=69%, milk=40%, whereas strawberry=34%, artificial food coloring=13%. The majority of Ped physicians (47%) responded that skin prick testing for food or inhalant allergens is not accurate or reliable until 3 years old. Of all physicians, 58% answered that medications may interfere with serum IgE results and 38% felt serum IgE testing was a useful screening test to detect the presence of food allergy. Our survey identified substantial knowledge gaps regarding common A/I conditions among IM and Ped faculty and residents at academic medical centers. Improved education and quality improvement strategies are needed to help provide the most accurate and up to date medical care for patients. MO; 3. Lynbrook, NY; 4. Houston, TX; 5. Springfield, NJ. Rationale: The purpose of this study was to evaluate the prevalence and severity of dry eye disease (DED) in a consecutive series of patients presenting to an allergy practice. Methods: 68 consecutive patients were tested for tear hyperosmolarity and polled for binary DED symptoms: fluctuating vision, lens discomfort, light sensitivity, watery eyes, tired eyes, redness, burning, itching and grit. Data were compared to 9,407 subjects polled nationally across ophthalmologist and optometrist practices. Results: Of the patients reporting to the allergy clinic, 42.6% were highly symptomatic (reporting ≥ 5 symptoms), while 15.8% reported ≥ 5 symptoms nationally. However, only 37.5% of the symptomatic subjects were hyperosmolar in the allergy clinic, compared to 51.6% nationally. Although a smaller prevalence, the hyperosmolar subjects presenting to the allergy center displayed a significantly higher severity, averaging 337±28 mOsm/L vs. 323±16 mOsm/L nationally (p < 0.0001). Non-DED subjects were clinically identical, averaging 295.7 mOsm/L vs. 297.8 mOsm/L nationally, which ruled out the possibility of instrumental variation or analytical drift accounting for the difference in severity. These data suggest that dry eye in the presence of allergy may be more severe than dry eye alone, and that symptoms alone were insufficient to determine diagnosis. Conclusions: There is a significant overlap in symptoms of both allergy and dry eye disease. Objective testing can help differentiate between the two etiologies. Further, concomitant DED in the presence of allergy may be more severe than DED alone. C.S. Barnes * , F. Pacheco, R. Allenbrand, L. Gard, C. Ciaccio, Kansas City, MO. Introduction: Exposure to cigarette smoke is a major asthma trigger and has a detrimental impact on pediatric asthma. Allergists frequently estimate pediatric exposure to cigarette smoke through questions directed to parents or care givers. To evaluate the relationship of reported indoor smoking to actual levels of nicotine measured in house dust we performed the following studies. Methods: Dust was collected from homes enrolled in the Kansas City Safe and Healthy Homes Project. Project enrollees were asked a series of questions concerning smoking in the home including the number of persons in the home who smoke and if anyone was allowed to smoke inside the house. A convenience sample of 58 homes containing 0 to 5 individuals who declared they smoked cigarettes was selected for analysis. For all samples, nicotine was extracted from 0.2 grams of dust and analyzed by immunoassay using commercially available reagents. For conformation in one-third of samples nicotine was extracted from 0.5 grams of dust and analyzed for nicotine by gas chromatography mass spectroscopy (GCMS). Results: Enzyme immunoassay results indicated nicotine values for the 58 dusts ranged from 0 to 330 nanograms per gram of dust. GCMS analysis results ranged from 0 to 340 nanograms per gram of dust. Correlation between the two methods was strong (r = 0.90). The lower limit of detection for the immunoassay was calculated at 4.0 nanograms per gram of dust and for the GCMS at 100 nanograms per gram of dust. Dustborne nicotine concentrations were greatest in homes where up to 3 declared smokers resided. The highest nicotine concentrations came from houses with declared smokers who smoked in the home. Dustborne nicotine concentrations in homes where smoking was not permitted inside the house ranged from 0 to 50 nanograms per gram of dust. Conclusion: Nicotine concentration in house dust can be measured by commercially available immunoassay methods. Homes where smoking was permitted had higher dust nicotine than homes where smoking was not permitted. However, homes where smoking was declared not permitted were not without nicotine in house dust. K.M. Maples * , T.C. Burch, A.M. Perkins, K.M. Cunnion, N.K. Krishna, H. Minto, L.K. Willis, J.O. Nyalwidhe, Norfolk, VA. Introduction: Currently there is no noninvasive assay to evaluate for the possibility of eosinophilic esophagitis (EoE) in patients with suggestive symptoms. The current standard of diagnosis and monitoring is endoscopy but this is an imperfect approach. Endoscopy is invasive, esophageal epithelial eosinophilia is not uniform, there is not a standardized high-power field (hpf) size and current diagnostic thresholds for eosinophils per hpf correlate poorly with clinical measures of disease. Identification of a truly noninvasive biomarker for EoE and a standardized, reliable assay is an important unmet medical need. Methods: Urine samples were obtained from patients with biopsy proven EoE (n=27) and from atopic controls (n=24) without eosinophilic esophagitis. We used a targeted Single Ion Monitoring LC-MS-MS strategy to quantify the levels of 3-Bromotyrosine (3-BrTyr) in creatinine-normalized urine samples. Descriptive statistical analysis was performed for 3-BrTyr level following stratification and compared using the Mann-Whitney U test. Results: The median urine 3-BrTyr level was 12-fold higher for all subjects with EoE (169.50 picograms 3-BT/400 mg creatinine/dL, IQR 40.74-412.86) compared with atopic controls (12.87, IQR 3.51-114.69 (P=.01)). In order to evaluate the potential utility of urine 3-BrTyr as a screening test we performed a subset analysis evaluating only newly diagnosed and untreated patients with EoE compared with atopic controls. The median urine 3-BrTyr level was 13-fold higher for untreated subjects with EoE (172.62, .60) compared with atopic controls (12.87, IQR 3.51-114.69 (P=.0459)). Using a threshold cutoff of 145 yields a sensitivity of 67% and a specificity of 79%. Thus, a 3-BrTyr level<145 yields a negative predictive value of 90.48% in atopic individuals. Conclusions: This proof-of-concept study demonstrates a reliable assay for urinary 3-BrTyr. Further, urinary 3-BrTyr has the potential to be a useful, noninvasive biomarker in the diagnosis and management of EoE. Introduction: Released in April 2014 by the Center for Medicare and Medicaid Services (CMS),the Provider Utilization and Payment Data Physician and Other Supplier Public Use File includes reimbursement information on 6000 different procedures/ services reimbursed to over 880,000 healthcare providers in 2012. We have analyzed this database hoping to better understand how Allergy/Immunology providers are contributing to the care of the Medicare population. Methods: Using the Physician and Other Supplier PUF Aggregate file, the average number of unique beneficiaries seen, total paid by medicare to each physician, and the average amount paid per unique beneficiary was calculated. These values were calculated for the other provider types for contextualization of the results across the healthcare field. We excluded any organizational provider types, non-MD/DO provider types, non-face-to-face diagnostic specialties, as well as specialties with less than 100 providers. Eighty nine providers types are included in the Physician and Other Supplier PUF with 53 provider types included in the final analysis. Focusing on Allergy/Immunology providers only, we calculated the average number of unique beneficiaries seen as new or established patients was also calculated from the database. Results: A total of 3119 Allergy/Immunology providers saw for an average of 160.4 (SD ±179.6) unique Medicare beneficiaries and collected an average of $52,762 (SD ± $103,425). Among Allergy/Immunology providers, the average amount paid per unique beneficiary was $313 (SD ±$449). Of the 53 provider types included in the final analysis, Allergists ranked 45 th in average number of unique beneficiaries seen, 40 th in average amount collected from CMS, and 16 th in average amount paid per beneficiary in 2012. Allergy/Immunology providers billed for an average of 35.2 (SD ± 31.9) unique Medicare beneficiaries for new patient visits (all types). The average number of established visit by A/I Providers were 123.34 (SD±178.5) for an average of 69.1 (SD±77.1) unique Medicare beneficiaries. Conclusions: The Physician and Other Supplier PUF provides a unique opportunity to better understand how Allergy/Immunology providers fit into the overall healthcare landscape. Allergy/Immunology providers can use this data better to understand how their practice compares to national norms. Background: Many authorities have recognized that gene-environment interactions likely play a key role in the developmental origins of allergy, now known to be mediated largely by epigenetic modifications. Prospective epigenetic studies, beginning in utero involving the collection of umbilical cord blood provides a window into the prenatal environment and a prospective sample to identify epigenetic markers that precede the development of allergy. Methods: The Kingston Allergy Birth Cohort (KABC) now includes over 400 children born in Kingston, Ontario, and surrounding rural areas with mother/child pairs recruited during the prenatal period, many of whom are between 18mo to 3y of age. 60 mother/child pairs thus far have returned to undergo skin testing (common environmental and food panel) and surveys completed regarding symptoms of potential atopic disease in the child as well as relevant home and dietary exposures in the first 2y of life. Some children have also been evaluated in local Allergists offices for suspected allergy. Results: Of 60 children evaluated thus far, 11 (18.3%) demonstrated at least one positive SPT: 8 had positive SPT to a food allergen, only 4 of whom had a clinical history of supporting food allergy, however. 5 children had at least one positive environmental SPT, 2 of which had symptoms suggestive of early asthma. On preliminary analysis, factors associated with, or trending with, positive SPT include furry pet ownership (P=0.038; Fisher's Exact Test), early-life second hand smoke exposure (P=0.1) and breastfeeding for less than 3 months (P=0.074). Conclusions: Data collection is ongoing. Our early high rate of atopy likely reflects interest-bias from parents concerned about allergies. Pets, second-hand smoke and breastfeeding will be further explored. We anticipate epigenetic analysis of cord blood from atopic children compared to age/gender-matched controls will provide a window into the prenatal environment and potentially identify biomarkers that precede allergic disease R. Kumar * , K. Singh, U. Mehto, S. Nagar, R. Prasad, Delhi, India. Background: Indoor air pollution is the third leading cause of disease burden in South East Asia as per Global burden of disease study 2010. Children are much more susceptible to household air pollutants as they spend most of their time indoors. The present study was thus planned to assess the effect of indoor air pollution on childhood asthma and/or allergic rhinitis. Methods: The present study is a cross sectional study to assess the difference in household air quality in houses having children (<17 years age) with or without asthma and/or allergic rhinitis in a rural setting (Village Khanpurjupti, Loni, Ghaziabad) of Delhi NCR region. Seventy households were selected in which none of the child had symptoms of asthma and/or rhinitis while the other seventy households had at least one child with asthma and/or rhinitis. A standard questionnaire was filled for all children enrolled in study, information was collected from parents of child during the study and potential pollutant sources were identified in their residence. PM 2.5 was measured by using UCB Particle and Temperature Sensor (UCB-PATS) from Berkley Air monitoring group, USA.VOCs were measured by using Phocheck Tiger PID, Ion Science limited, UK. The measurements of PM 2.5 and VOC were done at an interval of 10 minutes in each of 140 households for a day. Result: There were a total of 108 children (52 asthma alone, 20 allergic rhinitis alone and 36 with co-existent asthma and allergic rhinitis) in the 70 houses having children affected with asthma and/or rhinitis. The level of pollutants was found to be twice higher in affected children houses as compared to control houses. The average PM2.5 levels were significantly higher (9.68mg/m3vs 4.31mg/m3; p 0.011) in affected houses as compared to controls. Similarly VOCs levels were higher in these houses (2.222 ppm vs 1.161 ppm; p 0.128) but could not reach statistical significance. It was also seen that houses of children with asthma and/or rhinitis had higher sec-Introduction: Little is known about how quickly chronic idiopathic/spontaneous urticaria (CIU/CSU) patients respond to omalizumab treatment and for how long they maintain their response. We report patterns of treatment response from ASTERIA I and ASTERIA II (pooled). Methods: Subjects were randomized 1:1:1:1 and received placebo (PLB) or omalizumab 75, 150, or 300 mg (OMA75, OMA150, OMA300) every 4 weeks for 3 (ASTERIA II, n=322) or 6 (ASTERIA I, n=318) doses. Patients completed the Urticaria Patient Daily Diary (twice-daily), from which a daily Urticaria Activity Score (UAS) was calculated; the 7-day sum of UAS is given as UAS7. Response was defined in two ways: (1) well-controlled urticaria (UAS7≤6) and (2) complete response (itch and hive free, UAS7=0). The duration of response was defined as the percentage of the treatment period, during which patients experienced their responses. IRB approval and informed consent were obtained from all research subjects. Results: From baseline to week 12, UAS7≤6 was achieved at some point in time by 28% of PLB, 49% of OMA75, 56% of OMA150, and 73% of OMA300 patients; for UAS7=0: 13% of PLB, 26% of OMA75, 33% of OMA150, and 53% of OMA300 patients. During the 12-week treatment period, patients who reached a UAS7≤6 at some point in time, had this level of response for a mean percent of time of 45% (PLB), 42% (OMA75), 54% (OMA150) or 65% (OMA300); for UAS7=0 the mean percent of time was 34% (PLB), 29% (OMA75), 37% (OMA150), or 52% (OMA300). The median time to achieve UAS7≤6 was 10 weeks (OMA150) and 4 weeks (OMA300) and to achieve UAS7=0 was 10 weeks (OMA300). Less than 50% of patients achieved UAS7≤6 in PLB and OMA75 and UAS7=0 in PLB, OMA75, and OMA150, thus we did not calculate their median time to respond. Conclusion: More CIU/CSU patients in OMA300 achieved well-controlled or complete responses than patients in other treatment arms. OMA300 patients achieved their responses sooner and maintained them for longer than those in any other arm. These results provide additional insights about how CIU/CSU patients might respond to omalizumab therapy over time. These data can help healthcare providers by setting expectations of likelihood of response in CIU/CSU patients at different time points after omalizumab initiation. Number and percentage of patients who achieved the state of well-controlled urticaria or complete response by week and study arm. BSL = baseline; PLB = placebo; OMA = omalizumab, UAS7 = Weekly Urticaria Activity Score. Introduction: Patients with lower health literacy (HL) have poorer understanding and self-management of chronic diseases, including asthma. We hypothesized that parents/caregivers were an important source of HL for teenage students, which would be indicated by agreement between student and caregiver HL scores on standard instruments. Methods: HL status was assessed as part of Puff City, a randomized controlled trial of a web-based asthma education intervention in four rural Georgia high schools. Baseline sociodemographic data was obtained. Due to the small percentage of "other race," whites and other races were combined for analyses. Three validated instruments were used: REALM (Rapid Estimate of Adolescent/Adult Literacy in Medicine) and SILS (Single Item Literacy Screener). To examine agreement between scores, weighted kappa statistics were calculated. Bowker's test of symmetry was used to examine equality of disagreement in scores. Differences in REALM and SILS scores were examined with t-and chi-square tests. Results: 243 students and 203 caregivers completed HL assessments yielding 198 paired observations. The students attended grades 9-12, were 60.6% female, 72.7% African American (AA), with mean age of 15.3 (SD 0.9) years. Parent education ranged from less than high school diploma (19.1%) to college graduate (24.0%). Significant differences of REALM scores were seen between AA and white students, 56.2 (10.6) vs 60.8 (6.8), p<0.0005, respectively. Agreement between student and caregiver REALM categories was poor (κ w =0.26) while disagreement did not significantly differ by scores or demographic variables. There was significant disagreement between student and caregiver scores on SILS and this differed by caregiver race, with students having higher scores when their caregiver was AA. Agreement in SILS scores between student and caregiver was poor for both caregiver race groups, but whites/other race caregivers had a higher weighted kappa (κ w =0.31) than AA caregivers. Conclusion: The poor agreement between student and caregiver HL scores suggest that teens do not primarily acquire HL from caregivers. Consistent with previous adult literature, there appears to be a disparity in adolescent HL between AA and whites. The lower HL among AA teens suggests that they are likely to require more physician effort to become proficient in asthma self-management. Introduction: The aim of this study is to summarize and visualize the utilization and cost relational system between providers for pediatric asthma care in the Medicaid system using large patient-level claims data. We investigate an inferential and graphical approach for uncovering patterns in the healthcare utilization within the Medicaid system. We pilot this study for pediatric asthma patients in Georgia and North Carolina using the 2009 patient-level claims data. Methods: We apply sequence clustering analysis (SCA) to classify patients according to their utilization profiles, and for each cluster, we derive a probabilistic network of care where the probabilities are determined based on the patient-level utilization across providers of different types. The underlying utilization networks are the basis in deriving the cost relational system between providers. Results: We use the Markov chain representation of the utilization profiles to visualize the network model for each profile as derived from the SCA with three profiles. Profile 1 is dominated by patients primarily utilizing Emergency Department (ED) care services with a small proportion referred to primary care. Profile 2 is dominated by patients primarily utilizing primary care once or multiple times with a small proportion visiting ED. Profile 3 has a high variation in utilization. Among the children with asthma in the 2009 Medicaid system of Georgia, about half of the patients utilize primary care whereas the other half either seek care through ED or from multiple providers. The cost analysis shows that the distribution of cost-per-visit for ED visits is similar across the three profiles with a higher median for the ED-care prevalent profile. The costs for other provider types have similar symmetricallyshaped distribution but with some differences in the median across all three profiles. Conclusion: It is not surprising to find that ED visits contribute to a large portion of the cost for pediatric asthma care where most of the ED costper-visit values are in the range of other care visit types suggesting that many ED visits are for routine care. However, we also find that about half of the Introduction: Reslizumab (RES), a humanized anti-human interleukin-5, is in development for patients with moderate to severe persistent asthma with elevated eosinophils (EOS). We compared patient-reported outcomes (PROs) and safety of RES vs placebo (PBO) in patients with asthma and elevated EOS. Methods: This multicenter, double-blind, 16-week study (NCT01270464) included patients (N=311), 12-75 years of age, with uncontrolled asthma on at least medium-dose inhaled corticosteroids with/without additional controllers, Asthma Control Questionnaire score ≥1.5, and blood EOS level of ≥400/mL. Randomization was to intravenous RES 0.3 mg/kg (n=104) or 3.0 mg/kg (n=106) or PBO (n=105) Baseline clinical characteristics were similar among treatment groups. At 16 weeks, RES 0.3 mg/kg and 3.0 mg/kg improved AQLQ from baseline (mean treatment difference vs PBO 0.278 and 0.359, respectively). The difference between RES 3.0 mg/kg and PBO was statistically significant (P=0.0241). The percentage of patients with a MID in AQLQ at week 16 was 59%, RES 0.3 mg/kg; 64%, RES 3.0 mg/kg; and 48%, PBO. The difference between RES 3.0 mg/kg and PBO was statistically significant (P=0.0189). RES-treated patients had statistically significant (P≤0.016) ASUI improvements vs PBO (mean treatment difference vs PBO: 0.51, RES 0.3 mg/kg; 0.47, RES 3.0 mg/kg). Most (86%) patients received 4 complete infusions of RES. Incidence of adverse events (AEs) was similar among treatment groups. Serious AEs for RES occurred in the 3.0 mg/kg group (1 patient with pneumonia, road accident/rib fracture, and asthma exacerbation; 1 patient with sinusitis; 2 patients with asthma exacerbations). AEs led to discontinuation in 10%, <1%, and 6% of PBO, RES 0.3 mg/kg, and RES 3.0 mg/kg patients, respectively. Conclusions: In patients with uncontrolled asthma and elevated blood EOS, 4 monthly doses of RES were well tolerated and associated with improvements in PROs of quality of life and symptoms. This study was sponsored by Teva Pharmaceuticals. Background: Current models for calculating asthma related risk require clinical parameters or administrative data. Often, programs that conduct disease management, community outreach and environmental assessment do not have access to either. The aim of this study is to develop a simple risk stratification model to help allocate limited resources to children with high risk asthma. Methods: This is an IRB approved retrospective study of 11,915 patients <18 years seen for an asthma diagnosis at one of 23 outpatient clinics at our institution between 1/1/10 and 5/31/12, regardless of whether asthma was the principal diagnosis (PD). The frequency of asthma related acute care visits (ACVs) in the 12 months prior to the outpatient visit was determined and a risk score calculated based on ACV type (ED/UCC visit=1 point, inpatient admission=2 points, PICU admission=3 points). The total risk score was used to create four risk categories: Very Low (0 points), Low (1-2 points), Medium (3-6 points) and High (≥7 points). The odds of an asthma related ACV in the 12 months following the outpatient visit were then compared across risk categories using logistic regression. Results: Our study sample included 27,083 outpatient visits representing 11,915 unique patients. Of these visits, 74% were Very Low risk, followed by approximately 19%, 6% and 1% for Low, Medium and High risk, respectively. Eighty-two percent of High, 57% of Medium, 36% of Low and 11% of Very Low risk patients had an asthma related ACV in the 12 months following the outpatient visit. After accounting for clustering of visits by patient, the odds of a future ACV was significantly higher for High risk (odds ratio (OR)=36.8; p<0.001), Medium risk (OR=10.6; p<0.001) and Low risk (OR=4.5; p<0.001) patients, when compared to Very Low risk patients. The odds of a future ACV was significantly higher for High (OR=8.2; p<0.001) and Medium (OR=2.4; p<0.001) risk patients, as compared to Low risk patients. High risk patients had 3.5 times greater odds of having a future ACV than Medium risk patients (p=0.001). Conclusion: This scoring algorithm effectively stratified asthmatic patient risk for future asthma related ACVs. The potential utility and scope of this model could be significant in the allocation of asthma resources to those who need them most, particularly when access to detailed clinical data is limited. Introduction: Early recognition of severe combined immunodeficiency (SCID) is integral in the adoption of timely life-saving interventions, like hematopoietic stem cell transplantation for an infant born with the disorder. Population-based, inexpensive, and sensitive newborn screening (NBS) for SCID involves measurement of T-cell receptor excision circles (TRECs) via a quantitative polymerase chain reaction (qPCR) assay. Here we summarize the results of Iowa's one year SCID NBS pilot program. Methods: A TREC qPCR assay using DNA isolated from dried blood spots was developed by the State Hygienic Lab and ran on all infants born in Iowa during the June 3, 2013-June 31, 2014 pilot year. Samples with initial abnormal results were repeated and immunophenotyping was performed on all infants with abnormal results. Results: Of 43,930 infants screened, 13 were presumptive positive for SCID; one remained indeterminate on repeat screening. Threshold for abnormal results was revised twice during the pilot. Of these 14 infants, 6 were identified in the first month of the pilot and 8 were identified after cut-offs were revised in February 2014; 10 (71%) were male, 8 (57%) were in an intensive care unit, 3 (21%) were Hispanic, 3 (21%) were infants of diabetic mothers and 2 (14%) had congenital heart disease. Immunophenotyping was without significant Tcell lymphopenia ( CD3 < 1500 cells/ml, or absence/marked reduction in CD4 naïve T cells [CD4/CD45RA] < 5% of total CD3 T cells) in 11 infants, including one with 22q11.2 deletion syndrome. Significant T-cell lymphopenia was found in 3 (21%) infants; however, T-cell function (assessed as lymphocyte proliferation to phytohemagglutinin) was normal and none was found to have SCID. Three infants with significant T-cell lymphopenia underwent additional testing and clinical outcomes were monitored. One infant was diagnosed with Jacobsen syndrome with associated cytopenias, one with 18p deletion syndrome and complex congenital heart disease and one with no causative genetic mutation yet identified. Conclusion: In the first year of Iowa's SCID NBS program no SCID babies were identified; however, a variety of other conditions with Tcell lymphopenia were found. This finding is similar to that of other states reporting TREC NBS and may help to improve our understanding of other conditions that present with T-cell lymphopenia. S.E. Henrickson *1 , J. Heimall 2 , S. Jyonouchi 2 , 1. Haddonfield, NJ; 2. Philadelphia, PA. Introduction: TREC based NBS identifies newborns with asymptomatic SCID and allows HSCT with lower morbidity, mortality and cost. TREC NBS started in PA statewide July 2013, but hospitals can opt out. We review the cases of 4 PA born infants who recently presented: 2 via positive NBS and 2 who were born in hospitals not participating in TREC NBS and developed opportunistic infections. Results: Patients identified by positive NBS: Pt 1: Outpatient phenotyping showed T-B+NK-SCID; confirmed as X-SCID. Prophylactically admitted for 9d due to sibling illness. Readmitted DOL26 for 48h for unconditioned MRD PBSC transplant (PBSCT) . Readmitted with emesis and blood streaked stools (15d), asymptomatic CMV and EBV reactivation (48h). Total admissions 28d. Now well day+106. Pt 2: Outpatient phenotyping demonstrated T+B-NK+; found to have RAG1 mutation. Admitted DOL120 for myeloablative conditioned UCB transplant (UCBT), hospitalized 39d, including transplant associated microangiopathy. Readmitted with fever (7d), poor weight gain (6d November 8-9, 2014 georgia world congress center atlanta, georgia Introduction: Pompe disease (PD) is an inherited disorder caused by a deficiency of the lysosomal enzyme alpha-1,4-glucosidase and characterized by accumulation of lysosomal glycogen in many organs, including the heart and skeletal muscle, leading to myopathy. Patients with infantile-onset PD can develop severe cardiomyopathy, which if left untreated, may result in death within the first year of life. A recent breakthrough treatment with recombinant enzyme replacement has significantly improved patients' survival, but is often complicated by the development of enzyme specific antibodies precluding further administration. Here we report a case of successful desensitization to alglucosidase alfa in an infant who previously developed a systemic allergic reaction during enzyme replacement therapy. Methods: Specific IgE to alglucosidase alfa was obtained and reported to be negative. The patient underwent desensitization using our protocol starting approximately at a 1:10,000 dilution of the therapeutic dose, with doubling of the dose every 15 minutes until the cumulative dose approximated the therapeutic dose (See Table 1 ). Results: Patient was a 7 week-old neonate with infantile-onset PD receiving weekly intravenous infusions of alglucosidase alfa at 20mg/kg. He developed a diffuse urticarial rash, respiratory distress with wheezing, and periorbital and oral angioedema approximately 5 minutes into his 4th infusion. The following day, we proceeded with desensitization. He developed two generalized cutaneous allergic reactions during dose titration, but was able to complete the protocol. He was continued on dose escalation weekly and was eventually able to transition to his former outpatient infusion protocol. Discussion: The incidence of anaphylaxis from administration of this medication has been reported in the range of 1-14%. It is unclear if these cases are truly IgE vs. non-IgE mediated. Our patient's clinical history and cutaneous reactions during his desensitization suggest an IgE mediated mechanism. The specific IgE blood sample was obtained within hours of his anaphylactic reaction and thus may have caused a false-negative result. We report our case to illustrate a protocol that was effective in inducing temporary drug tolerance to alglucosidase alfa, allowing continuation of lifesustaining treatment for this patient. Background: Food Protein Induced Enterocolitis Syndrome (FPIES) is a rare, but increasingly prevalent non-immunoglobulin E (IgE) mediated gastrointestinal reaction to food proteins. It is outgrown in the majority of children by 30 months of age, and does not show predominance for atopic indi-viduals or families. We present the case of a 7 year old girl with severe atopic dermatitis that was controlled with topical steroids and the soak and seal method. She had a history of multiple food reactions consistent with IgE mediated food allergy. At 18 months, she ingested sweet potato, resulting in profuse vomiting that was diagnosed by her pediatrician as anaphylaxis. Methods: Skin prick and ImmunoCapp testing was performed followed by a food challenge to sweet potato in an observed medical setting. Results: Skin prick testing with commercial extracts revealed sensitivity to multiple foods including peanut, tree nuts, milk, egg, shellfish and wheat but was negative to sweet potato. The patient's total serum IgE was 4504 kUA/L, and ImmunoCapp to sweet potato was 2.73. In comparison, her milk IgE was >100. Based on these results an oral food challenge was performed. There was no immediate reaction (within 20 minutes) to sweet potato given in increasing doses over 2 hours. One hour after the full serving, she began to vomit profusely, and continued to vomit over the following 3 hours, despite treatment with diphenhydramine, IM epinephrine and IV ondansetron. IV fluid resuscitation was also performed. A complete blood count obtained during the reaction revealed a significantly elevated neutrophil count of 17,100, compared to baseline of 2,800. She also had a normal tryptase. These results are consistent with an FPIES reaction to sweet potato. Conclusion: FPIES is generally considered in the differential diagnosis when infants and toddlers present with acute reactions to food ingestion, but not in older children. This child posed a further diagnostic challenge given her concomitant IgE mediated food allergies to multiple foods. This unusual case, never reported in the literature, demonstrates the potential for distinct pathogenic mechanisms in children with multiple food sensitivities. Physicians should consider in the differential diagnosis and be prepared to treat FPIES in any child with a history of profuse vomiting after ingestion of certain foods. A. CaJacob *1 , C. Adkins 2 , M. Descartes 1 , C. Allen 1 , J. Anderson 1 , 1. Birmingham, AL; 2. Hoover, AL. Introduction: Morquio A Syndrome (also known as mucopolysaccharidase type IVA deficiency) is an autosomal-recessive lysosomal storage disease characterized by a deficiency of the N-acetylgalactosamine-6 sulfatase enzyme. Until the recent FDA-approval of elosulfase alfa (Vimizim™) enzyme replacement therapy (ERT) in February 2014, treatment of this progressive, multiorgan disease had been symptomatic care. In pre-market clinical trials it was determined to carry a 7.7% risk of anaphylaxis. We present a patient who was successfully desensitized to elosulfase alfa after an anaphylactoid reaction. Methods: An nine hour desensitization protocol was devised. We began with an approximate initial dose of 1/10,000, with doubling of the dose until the patient received her goal dose of 200mg. Results: A 54 year old female with Morquio A syndrome began elosulfase alfa ERT in October 2013. She tolerated weekly infusions without difficulty until March 2014 when after approximately 2.5 hours into her twenty-third infusion she developed a reaction characterized by fever to 102.7, tachycardia, hypotension, wheezing and hypoxia (O2 sat 75%). Reaction was treated with intravenous fluids, antihistamines, nebulized albuterol and methylprednisolone. She was referred to the Allergy service one week later for evaluation. Skin prick testing (patient and control) was negative to 1:1, 1:10 and 1:100 dilutions with good histamine control. The patient's intradermal testing was positive at a 1:10 dilution. The control's intradermal testing was negative at 1:10 and 1:1 dilutions. One month after her reaction, we proceeded with the above desensitization protocol. The desensitization was well-tolerated. After its completion, the patient resumed her weekly infusions at a slower rate without further reactions. Repeat skin testing four months from initial desensitization was negative at 1:10 and 1:1 dilutions. Conclusions: Vimizim™ is an FDA-approved ERT for Morquio A syndrome patients which carries an anaphylaxis risk. The immunologic basis of our patient's anaphylactoid reaction is unclear, particularly given the fever and the absence of any skin manifestations. After desensitization, she had conversion to negative skin testing. No antibody assay is currently available. To our knowledge, this case represents the only elosulfase alfa ERT desensitization in published literature. Anaphylaxis is a serious allergic reaction that may cause death. The present study examined the symptoms, triggers and demographic patterns of patients treated for anaphylaxis at a large tertiary care hospital in Riyadh, Saudi Arabia. The protocol was approved by the Institutional Review Board of the hospital. All the patients who were prescribed Epinephrine auto-injectors ( EA ) between 1/1/2010 and 31/12/2011 were included in this study. 238 new patients were identified using hospital pharmacy database and the medical records of these patients were reviewed. To date, Epinephrine auto-injectors ( EA ) are available at very few hospitals in Saudi Arabia. Since our hospital is the main tertiary care hospital with allergy&immunology service in the Kingdom, these data may reflect Anaphylaxis presentations in our region. The mean age at the time of first EA prescription was 22.9 years, (2years to 73 years). SD +/-18.2 Female to male ratio was 52:48. 54% for subjects were less than 18 years of age. 18 % were Non Saudis living in the Riyadh region. Most of these were employees of the hospital or their dependents. Although most of the signs and symptoms were similar to other published data, there were some differences observed in our population. Urticaria and angioedema was the most common presentation accounting for 70% in adults and children followed by shortness of breath in 33% in adults and 23% in children. Food was the commonest trigger for anaphylaxis with Tree Nuts and Egg the most frequent, followed by Sesame and Peanut. Drug Allergy was also a common trigger, with Penicillins and NSAIDs being the commonest. Regarding Insect allergy, Samsum Ant was the commonest trigger in our population. This is the first such study on Anaphylaxis in Saudi Arabia. While there are many similarities, some of the presenting signs, symptoms, and triggers of anaphylaxis are significantly different in our population than in others. While treating Anaphylaxis, we should be mindful of these differences. This improved understanding should help reduce the morbidity and mortality associated with Anaphylaxis in our region. Introduction: DRESS is a severe and potentially fatal drug reaction characterized by fever, rash, lymphadenopathy and visceral organ damage. We describe the case of a patient with Phenytoin induced DRESS presenting as eosinophilia and laryngeal edema. Case Report: The patient is a 59 yo AA Female with Hypertension, Type 2 Diabetes, Hyperlipidemia, Hypothyroidism and a history of Cerebral Vascular Event and subsequent seizure. She has no history of previous angioedema. Her home medications include alendronate, atorvastatin, cholecalciferol, folic acid, insulin, levothyroxine, lisinopril, omeprazole and phenytoin which was initiated 2-3 months prior. Initially she presented to the ED for fever and an urticarial and maculopapular rash on her neck, chest and abdomen. She was placed on antimicrobial therapy for presumed UTI for which she took for two days before returning to the ED with worsening rash and desquamation. At this evaluation she was given oral prednisone burst. Five days following completion of steroids she again presented to the ED with fever and facial edema. She was emergently intubated by ENT for oropharyngeal edema. She was started on Dexamethasone 8mg q8 for edema and a CT obtained demonstrated extensive angioedema of her tongue and larynx with cervical lymphadenopathy. On exam she had no rash, but was noted to have desquamation on her torso and arms. Her initial laboratory evaluation showed WBC 21,700 cells/mm 3 , AEC 10,200 cells/mm 3 , normal C3 and C4. With three days of systemic steroids, her AEC improved to 8650 cells/mm 3 . The edema was not improved and she developed hepatic injury (AST 215, ALT 242, Alk Phos 1201) with mild hepatitis suggested on ultrasound. Phenytoin and Lisinopril were discontinued as her picture was consistent with DRESS. She quickly improved and was extubated 4 days following Phenytoin discontinuation. Discussion: Facial edema is common in DRESS however oropharyngeal and laryngeal involvement have not been described. Tongue edema has been reported previously in a patient concomitantly on an ACE inhibitor, as was our patient. This may implicate a synergistic effect of ACE inhibitors and DRESS presentation. To our knowledge this is the first report of DRESS presenting with laryngeal involvement and intubation. Introduction: Patients with suspected peanut allergy (PA) come to medical attention following an adverse food reaction or in the course of in vivo or in vitro testing. An IgE level of 15 kU/L or above has been suggested as 95% likelihood of PA, however the majority of patients sensitized to peanut have an IgE level below this cut off. As some patients avoid peanut after initial symptoms, a negative history of systemic reaction to peanut has uncertain predictive value. This creates a diagnostic dilemma for clinicians who wish to recommend avoidance, consumption or the option of a food challenge. At what lower limit of peanut IgE should component testing be considered? Peanut component testing provides a more certain decision point; sensitization to peanut seed storage proteins (SSP), Ara h 2 in particular, correlates well with symptomatic peanut allergy. Based on food challenges, Codreanu proposed an IgE threshold for Ara h 2 of 0.23 kU/L for identifying peanut allergic subjects, Nicolaou and Custovic proposed 0.35 kU/L as a threshold. This study considers the peanut IgE level that may be used as a decision point for reflex component testing. Methods: This prospective study utilized sera with de-identified patient health information. Samples from 25 peanut sensitized subjects, age 12 years and younger with specific IgE 0.35-15 kU/L, were assayed for peanut SSP Ara h 1, Ara h 2, Ara h 3, for PR-10 Ara h 8, and lipid transfer protein (LTP) Ara h 9. Results: 76% of the subjects were sensitized to peanut SSP, 56% to Ara h 2, 20% were sensitized to PR-10, Ara h 8 and 12% to LTP, Ara h 9. Ara h 2 of at least 0.35 kU/L was seen at the lowest extent of the peanut IgE data set. In one sample a value for Ara h 2 of 0.44 kU/L corresponded to a peanut IgE of 0.41 kU/L. Within the complete data set it was not possible to predict sensitization to Ara h 2 or SSP based on peanut IgE as the profile may contain IgE to SSP or cross-reactive proteins. Conclusions: There is not a lower peanut IgE threshold for component testing and it is not possible to predict the component profile based on the peanut IgE alone. Peanut IgE values <15 kU/L may be associated with values of Ara h 2 exceeding peanut challenge decision points. Component testing has improved in availability and affordability. Medical decision-making based on the component profile allows a more accurate risk assignment than decisions based on peanut IgE alone. Introduction: Fixed drug eruption is characterized by the sudden onset of a single or few circular macular erythematous-violaceous lesions, that could happen in a specific location repeatedly after exposure to a particular drug in 85 to 100 % of cases; drugs most often involved are sulfonamides, NSAIDs, tetracyclines, paracetamol, anticonvulsants and other anecdotal drugs as antifungal. There are five clinical forms: a) classic characterized by an asymmetric and erythematous pigmented shape b) symmetrical form with erythematous unpigmented plaques, c) is minor or individual lesion, d) Disseminated bullous form, e) linearly. The responsable drug was identified through patch test. Case Presentation: Thirty eight years old female presents multiple slim, erythematous, assymetrical lesions 24 hours after treatment with Trimethoprim -Sulfamethoxazole located in the back with regular discoid margins with slow progressive involution. After suspension of TMP-SMZ exposure the patient preserved a lesion in the calf with residual pigmentation. She had a history of previous similar episodes with unspecified drug exposure, 6 weeks later fixed drug eruption by TMP-SMZ was confirmed with patch testing. Discussion: Fixed drug eruption has a better prognosis than other drug induced skin lesions and is considered a form of delayed hypersensitivity mediated by T cells CD8 +, the disease could determine important physical and psychological impairment due to discomfort and cosmetic appearance, thereby physicians should be alert of this possibility to avoid subsequent recurrent episodes. The literature indicates that patch testing is less dangerous than oral challenge to identify the responsible drug with positivity of 23.6% to 20%, which proved effectiveness in the presented case, thereby this diagnostic tool should be considered as a first step approach for fixed drug eruption. Introduction: Rashes are common in patients receiving multiple medications. Most rashes are benign but some can be life-threatening and require urgent identification & discontinuation of the culprit which can be difficult. Case: A 49-y-o AA man was transferred from another hospital because of severe generalized rash started 2 wk earlier as pustules on legs then spread as bullous-pustular all over his body except the face; accompanied by on-off fever up to 101F. He did not improve on TMP/SMX & rifampicin or on IV vancomycin, piperacillin-tazobactam & prednisone 20 mg daily. Review of systems revealed HTN, RA, gout, obstructive sleep apnea & peptic ulcer; on amlodipine, olmesartan, aliskiren, nebivolol, clonidine, methotrexate, allopurinol, modafinil, pantoprazole, iron and folic acid. He was started on adali-mumab (Humira) for his RA 7 wk & 1 wk before the rash started. On presentation, his VS & PE were normal except for generalized ulcerated bullous pustular eruption without facial or mucous membranes involvement. Lab findings: WBC 23000 with 95% N, ESR 81mm/hr, CRP 45mg/dl & normal CMP. Lesion smear showed neutrophils with negative culture & no fungi or AFB. Blood culture was also negative. He was treated with hydrotherapy, wound care, and IV methylprednisolone 60mg daily & diphenhydramine 25mg q 6 hr. Biopsy showed dermal microabscesses & leukocytoclastic vasculitis consistent with pyoderma gangrenosum (PG). Methylprednisolone was increased to 1 g daily for 5 d then switched to PO prednisone 60 mg daily. Further evaluation showed positive ANA with no specific pattern, and negative RF, CCP, Ds-DNA, anti-Smith Abs, ANCA & PSTPIP1 mutation. Serum & urine electrophoresis and C3 & C4 were normal. Serum Humira Abs (IgG) was 15.8 U/ml. The rash gradually improved until completely resolved over 3 wk while prednisone was being gradually tapered to 20 mg daily on discharge. Conclusion: PG was diagnosed based on biopsy & negative workup for other bullous pustular disorders. Humira was the most suspect because it was the most recent medication received, elevated serum Humira IgG Abs, and resolution while on all his other medications. This may be the first reported PG case caused by Humira. PG is uncommon neutrophilic dermatosis affecting mainly young adults; > 50% of cases have an associated systemic inflammatory disease (e.g., IBD, hematologic or rheumatologic). Most patients achieve remission on immunosuppressants but relapse can occur. Introduction: Drug reaction with eosinophilia and systemic symptoms (DRESS) is a severe acute drug reaction with a highly variable clinical presentation. It is characterized by a polymorphic skin eruption associated with fever, eosinophilia, lymphadenopathy and the involvement of multiple organs (liver, kidney and lung) along with a long latency (two to eight weeks) between drug exposure and disease onset. Methods: We evaluated a 14-year-old boy with history of allogenic peripheral blood stem cell (PBSC) transplant for acute myelogenous leukemia (AML). He presented to Allergy clinic for evaluation of rash and eosinophilia. Results: The patient received a matched unrelated donor PBSC transplantation for high-risk AML 7 months prior to his presentation. He had history of chronic skin graft-versus-host disease requiring longterm therapy with prednisone. Daily medications also included lisinopril, acyclovir, cetirizine, vitamin D, omeprazole and sulfamethoxazole/trimethoprim. After 6 months of therapy, prednisone was slowly weaned to a physiologic dose of hydrocortisone. Within a week of stopping prednisone he developed intermittent low-grade fevers and an erythematous and pruritic rash on his legs that progressively worsened despite treatment with topical triamcinolone 0.1% ointment and oral diphenhydramine. Over a period of 2 months he had 3 hospital admissions for work-up of fever and worsening rash. Blood cultures were negative. During his 3 rd admission new-onset eosinophilia and worsening transaminitis were noted. Based on these findings he was diagnosed with DRESS. Therapy with sulfamethoxazole/trimethoprim was stopped and he received 7 days of prednisone resulting in improvement of his rash. Within a few days of completing the prednisone course he developed recurrence of his fever and rash and was readmitted. A skin biopsy confirmed a drug reaction. He was initiated on a 3-month course of prednisone with complete resolution of his symptoms. Conclusions: Our case report highlights the difficulty of diagnosing DRESS syndrome. Though the syndrome is known for its long latency between exposure and symptom onset, the diagnosis and symptoms in our patient were likely initially masked by his chronic prednisone use. Introduction: Mycophenolate mofetil (MMF) is increasingly being used in place of azathioprine in organ transplantation, as it is associated with less bone marrow suppression, fewer opportunistic infections, and a lower incidence of acute rejection. MMF, a purine nucleotide analogue, blocks lymphocyte proliferation by inhibiting guanine nucleotide synthesis in lymphocytes. Methods: A case report Results: A 46-year-old African-American female with systemic lupus erythematosus (SLE) induced dilated cardiomyopathy and end stage renal disease underwent double heart-kidney transplant. Following the transplant the patient was placed on azathioprine, tacrolimus and methylprednisolone. MMF was not included in her immunosuppressive regimen due to a previous episode of oral pruritus within minutes after the first dose for lupus nephritis approximately 10 years ago. A second trial of MMF three weeks after the first resulted in urticaria, pruritus and angioedema. MMF was discontinued and her symptoms subsided within 3 days.Allergy and Immunology was consulted to evaluate for possible reinitiation of MMF. Skin testing to MMF was performed, however the patient had a blunted response to the histamine control. Given the urgency of the situation and inability to interpret our skin tests, we designed a novel 12step desensitization protocol (Table I) adapted from current Drug practice parameters 1 . In an intensive care unit setting, an oral desensitization with MMF was performed with diphenhydramine 25 mg IV and famotidine 20 mg IV given as premedications. She completed the desensitization without adverse reactions. Conclusion: The use of MMF has become standard practice in many solid organ transplant recipients due its efficacy and favorable risk profile compared to other immunosuppressants. There has been a single case report of successful MMF desensitization 2 . However, this protocol did not follow current Drug practice parameters and was done over a three-day period. We report a successful desensitization to MMF in a double heart-kidney transplant recipient. Rationale: Although rare, amphotericin adverse reactions may occur. Methods: Report of a case and review of the literature. Case Presentation: A 39 year old male with disseminated cryptococcus meningitis infection after exposure to deceased birds and feces presents with rash. His only new medications were flucytosine and liposomal amphotericin. The rash presented as itchy, slightly raised, diffuse erythema most consistent with an urticarial lesion on bilateral knees on Day 7 of antifungal therapy. No lip/tongue swelling, difficulty breathing, abdominal pain, or vomiting. No oral ulcers or skin peeling. Flucytosine was continued and liposomal amphotericin was held to help further determine the causative agent. On Day 8 of antifungal therapy, he tolerated flucytosine. The rash was nearly resolved. On Day 9, flucytosine was continued and liposomal amphotericin re-initiated. 1 hour into liposomal amphotericin infusion, he developed a similar rash on bilateral knees. Thirty minutes after the infusion, he developed chest tightness lasting 30 minutes. On Day 10, he underwent liposomal amphotericin desensitization with an initial concentration of 1 ng/ml infused at 1.67 ml/min rate with 3 fold increments in concentration every 30 minutes for a cumulative dose of 300mg. There were a total of 14 increments. 4 hours after completing the desensitization, he developed a similar rash on his bilateral knees, left calf, and right foot. On Day 11, rash was nearly resolved. On Day 11 evening, he underwent a conventional amphotericin (without liposomal component) desensitization with a similar protocol except to a cumulative dose of 0.7mg/kg (54mg). There were a total of 10 increments. Results: He was successfully desensitized to conventional amphotericin B after failing desensitization with liposomal amphotericin. Discussion: Amphotericin is the drug of choice for treatment of severe invasive fungal infections. The use of liposomal amphotericin has increased given the less adverse side effects. In review of the literature, there was a case of a child who had urticaria to liposomal amphotericin but tolerated conventional amphotericin. In patients who adversely react to liposomal amphotericin, one may consider desensitization to conventional amphotericin B, as the patient may be reacting to the liposomal component or to an epitope array of amphotericin that only gets presented by a liposomal formulation. Introduction: Catamenial anaphylaxis, a.k.a. cyclical anaphylaxis, is characterized by recurrent episodes of serious, possibly fatal, multisystem allergic reactions occurring at the time of menstruation. It is an uncommon clinical entity with few reports. Presentation: A 38-year-old Caucasian female patient with recurrent anaphylaxis: abdominal cramping, diarrhea, injection and swelling of the eyes, nasal congestion, wheezing, respiratory distress, throat closing. First episode occurred while she was working at a breakfast restaurant. She had worked there on and off for 4 years previously without any such issues. Continued to have recurrent episodes x 2 over the next three months, then every 4 weeks. Each time, the episodes resolved with oral antihistamine. The patient never used epinephrine and never sought medical attention after the episodes. No identifiable food or medication triggers. CBC and CMP within normal limits. RAST IgE positive for dog, cat, ragweed, total IgE 179. Baseline tryptase normal at 3.2; did not get acute tryptase drawn. No prior medical history other than asthma and allergic rhinitis. On questioning, patient noted that she had very good control of asthma symptoms except during her perimenstrual period, when she would have wheezing and dyspnea on otherwise normal exertion. Patient also noted that all anaphylactic episodes were occurring on/around Day 1 of menses and that she was completely asymptomatic in between. Patient was successfully treated with hormonal suppression of menses. Discussion: Catamenial anaphylaxis is a rare, possibly underdiagnosed clinical condition. It is a diagnosis of exclusion which can be identified after an appropriate evaluation, and when conditions such as mastocytosis and C1esterase inhibitor deficiency/dysfunction have been ruled out. No obvious pathogenesis identified, but possible theories include hypersensitivity to progesterone, hypersensitivity to estrogen, prostaglandins in menstrual fluid. There is no one universal method of treatment, as several of these pathways require different interventions. S. Joychan *1 , K. Dass 2 , 1. Kalamazoo, MI; 2. Chicago, IL. Anaphylaxis is an acute, potentially life-threatening systemic hypersensitivity reaction, resulting from exposure to an allergen. Classically, this occurs when an individual is exposed to an allergen, leading to sensitization and subsequent exposures result in an immunologic response. A 22-year-old female with a history of polysubstance abuse presented with anaphylaxis. The patient had a history of frequently presenting to the emergency room (ER) with vague complaints of abdominal pain, nausea, and vomiting usually after meals. No cause had been found for her symptoms, which were attributed to drug-seeking behavior. She presented to the ER one day prior to admission with mild uvular swelling, tachycardia, diffuse urticaria, and voice hoarseness. Her symptoms were attributed to an allergic reaction of unknown etiology. She was given intravenous (IV) methylprednisolone, IV famotidine, IV diphenhydramine, and two doses of intramuscular (IM) epinephrine. The patient was monitored and discharged when symptoms resolved. The next day, she presented again with dyspnea, nausea, and urticaria after consumption of garlic bread. She reported "outgrowing" a childhood peanut allergy. She also reported having recently started working at an Italian restaurant but denied workplace exposure to peanuts. She was unaware of any new exposures. IV methylprednisolone, IV famotidine, IV diphenhydramine and IM epinephrine were given, in addition to aggressive IV hydration. Tree nut panel confirmed allergy to almond, Brazil nut, cashew, coconut, hazelnut, macadamia nut, pecan, pine nut, pistachio, peanut, sweet chestnut, and walnut. Upon further questioning, the patient noted pine nuts on her garlic bread. All other allergy tests were negative. The patient received extensive counseling regarding her allergies and risk for anaphylaxis. She was provided with a prescription for an epinephrine auto-injector and recommended to follow up with an allergist outpatient. This case demonstrates the importance of taking a detailed allergy history and performing a thorough examination when presented with a case of anaphylaxis. Clinicians should be conscientious about recognizing vague symptoms that might precede an allergic reaction. This case also highlights the importance of differentiating signs and symptoms related to a patient's substance abuse from similar symptoms that can represent a true allergic reaction. PATIENT WITH DRESS SYNDROME. J.A. Mendez * , C. Acantilado, S. Nazario, San Juan, Puerto Rico. Introduction: Drug reaction with eosinophilia and systemic symptoms (DRESS) is a rare and life threatening delayed hypersensitivity reaction caused by exposure to certain medications. However, identification of the culprit drug may be challenging, particularly in patients receiving multiple medications. Patch testing is a well-known method used to identify the culprit drug in delayed Type IV hypersensitivity reactions. Here, we report the case of a pediatric patient in whom lamotrigine-induced DRESS syndrome was successfully confirmed through patch testing. Case description: A 5 year-old boy with epilepsy had been receiving valproic acid for over 1 year to control seizures. He had persisted with symptoms despite medical therapy and a follow-up electroencephalogram (EEG) still revealed abnormal electrical activity. A decision was made to change his antiepileptic therapy to lamotrigine 125mg daily. Two weeks after starting therapy with lamotrigine, the patient experienced the sudden onset of fever of 103° F and a diffuse maculopapular skin rash. Laboratory workup was remarkable for an elevated eosinophil count of 0.86x10 9 /L. A hypersensitivity reaction to lamotrigine was suspected and the medication was stopped immediately. A complete resolution of symptoms was observed after 2 weeks. He was referred to our clinics due to suspected drug allergy. Patch testing was performed to lamotrigine 30% in water and in petroleum jelly. Patch test reading at 72 hours revealed a positive result to lamotrigine in water. Conclusion: Patch testing of medical products is a useful tool in the evaluation of patients with delayed Type IV hypersensitivity drug reactions. In this case, patch testing was successfully used to confirm lamotrigine-induced DRESS syndrome in a pediatric patient with epilepsy. Positive patch test to lamotrigine 30% in water at 72 hours. Introduction: Drug reaction with eosinophilia and systemic symptoms (DRESS) is a drug-induced hypersensitivity reaction that includes skin eruptions, eosinophilia, lymphocytosis, lymphadenopathy and internal organ involvement. First line therapy includes high dose corticosteroids, however, patients with DRESS often require a slow, prolonged taper to prevent relapse. Here, we describe the case of a patient with DRESS effectively treated with mycophenolate mofetil as a steroid sparing drug. Case Presentation: Our patient is a 14-year-old white female, previously in good health. She developed a cough, malaise and sore throat treated at home with ibuprofen and pseudoephedrine. Her father also gave her one dose of amoxicillin left over from an old prescription. A few days later her symptoms subsided. Four weeks after the onset of the initial illness, she developed a flat pink rash, joint pains, fever, and cervical lymphadenopathy. Her symptoms continued to escalate until she became lethargic, had altered mental status and dyspnea. She was brought to the hospital hypotensive and required intubation. A CT scan revealed diffuse cervical, axillary, hilar, intra-abdominal, pelvic mesenteric and inguinal lymphadenopathy. She had leukocytosis of 80 K/ml, 20% bands, eosinophilia of 28 K/mL, lymphocytosis 29 K/mL, acute hemolytic anemia, thrombocytopenia, elevated creatinine, ferritin of 5500 ng/ml, and sIL2R 79,000 pg/ml. A bone marrow and lymph node biopsy showed no evidence of hemophagocytosis or malignancy. Workup for lupus, vasculitis, and infection was negative. She was diagnosed with DRESS. She received 1gm IV steroids for three days with remarkable recovery of pulmonary, renal and hepatic function and normalization of labs. She was transitioned to oral prednisone at 1mg/kg and started on mycophenolate mofetil 500 mg bid before discharge. Her steroid dose is tapered without relapse. Conclusion: DRESS is characterized by a profound immunologic response involving overproduction of multiple cell lines including T-cells, B-cells, eosinophils and production of abnormal antibodies. Mycophenolate mofetil is a non-competitive inhibitor of inosine monophosphate dehydrogenase that inhibits T and B Cell proliferation. To our knowledge, this is the first described case of using this agent to successfully treat a patient with DRESS. Background: Hymenoptera stings can cause allergic reactions in individuals sensitized to their venom. There is few epidemiological data on the prevalence of this type of allergy in children in México. The aim of this study is to know the prevalence of Hymenoptera sting allergy in school children of Monterrey, Mexico. Method: We conducted a transversal and observational study where hymenoptera allergy was surveyed among children attending to elementary school. The study was divided into two phases. First phase consisted in design and validation of a questionnaire to survey hymenoptera allergy. In the second phase, questionnaire was applied to parents whose children attended to any of the different elementary schools of the city. Finally, questionnaires were collected and submitted to analysis. The clinical reactions and epidemiological characteristics to ant bites, bee and wasp were investigated. Allergy to hymenoptera sting was correlated with personal history of atopy. Results: A total of 323 questionnaires were completed and selected for analysis. The age of the students ranged from 5-13 years (mean 9.6 ± 21) and 162 (50.2%) were female. Ninety-three children (28.8%) had a personal history of atopy and 23% had some clinical suspicion of atopy. Of the total sample, 217 children (67%) had a history of at least one wasp sting. The 10.5% of children had large local reactions and 6.5% systemic reactions. Anaphylaxis occurred in 0.9% of the children. We found no significant difference in the prevalence of allergy to Hymenoptera between atopic and non-atopic subjects (p = 0.09). Conclusions: The prevalence of large local reactions and systemic reactions found in this study was similar to what has been reported by other authors. However, the prevalence of anaphylactic reactions was lower than what has been reported in other epidemiological studies in the pediatric population. Introduction: Drug induced immune hemolytic anemia (DIIHA) is a rare but often severe cause of hemolysis that was first described in the 1950's. Currently, there are more then 125 different drugs known to cause DIIHA with cephalosporin and penicillin antibiotics being the most common agents. A diagnosis of DIIHA is made by a clinical history of drug associated hemolysis and positive serologic testing. Once DIIHA is identified, the causal drug should be discontinued, and avoidance of similar classes of medications is generally recommended since the cross reactivity between related medications is not well established. Methods: Case Report Results: A 43 year old female with cystic fibrosis (CF) and a history of life-threatening, piperacillin-induced immune hemolytic anemia was hospitalized with worsening productive cough and shortness of breath refractory to tobramycin, aztreonam, and azithromycin. Sputum cultures were notable for persistent pseudomonas aeruginosa infection sensitive to piperacillin/tazobactam (PT) and meropenem (MP); however, two years prior to admission, the patient developed severe hemolytic anemia with her hemoglobin reaching a nadir of 2.7gm/dL 5 days after starting PT. Laboratory evaluation at that time was significant for an elevated LDH and bilirubin, an undetectable haptoglobin, and a strongly IgG positive DAT. The patient was admitted to the ICU and required multiple blood transfusions but improved within a few days of stopping PT. Given the patient's history of DIIHA to piperacillin and serious nature of her current CF exacerbation, MP was deemed medically necessary for treatment, and informed consent was obtained. A 16 day graded dose challenge was performed starting at 1mg IV to a goal dose of 2 grams IV q8hrs without any evidence of hemolysis. The patient has continued to tolerate subsequent courses of MP without any adverse events. Conclusion: This is the first known report of a patient with a history of piperacillininduced immune hemolytic anemia tolerating meropenem through a cautious graded dose challenge. A. Blaziene 1 , N. Buterleviciute 1 , V. Paltarackiene 1 , K. Linauskiene 1 , L.M. DuBuske *2 , 1. Vilnius, Lithuania; 2. Gardner, MA. Background: Anaphylaxis is a life threatening condition. NSAIDs a most common pain relief medicine, may induced anaphylaxis. Methods: 70 patients (38 women (54.3%) and 32 men (45.7%) with anaphylaxis hospitalized in a Vilnius University Hospital from 2009 to 2012 were assessed. The mean age of patients was 47.31 ± 17.63 (range 20-83) years old. A total of 26 patients with drug-induced anaphylaxis were included in this study. The causes, symptoms and diagnosis of anaphylaxis were analyzed using WAO criteria. Results: NSAIDs were the main suspected cause of anaphylaxis for 10 (38.5%) patients. Other triggers of anaphylaxis were antibiotics (30.8%), local anesthetics (3.8%) and other drugs (26.9%). 9 cases of NSAIDs induced anaphylaxis fulfilled the second WAO criteria for anaphylaxis diagnosis and 1 met the third criteria. 90.0% of patients had cardiovascular symptoms, 70.0% had skin symptoms, 60.0% had respiratory symptoms and 40.0% had gastrointestinal symptoms. One patient experienced NSAID induced anaphylaxis twice. Provocation tests were performed and safe alternative NSAIDs were chosen for 2 patients. Conclusion: Drug induced anaphylaxis was most commonly induced by NSAIDS. Anaphylaxis manifestation typically involved the cardiovascular system, followed by skin and respiratory systems. Introduction: Cyclosporine is an immunosuppressive and immunomodulatory drug that acts selectively on T-cells by inhibiting calcineurin. It has been used in the treatment of a variety of T cell-mediated inflammatory and immunemediated skin diseases. Methods: A 40 year old Caucasian female was started on a beta adrenergic receptor blocker (metoprolol) for ischemic cardiomyopathy. On the third day, the patient developed a non-urticarial, pruritic and vesicular rash on her upper chest and extensor surface of her hands, along with angioedema of her face and fingers. The patient had no other systemic symptoms. The medication was discontinued and the patient responded to oral prednisone and hydroxyzine. After a few weeks, her cardiologist started her on a different beta blocker (carvedilol). The patient had a similar cutaneous reaction with angioedema on the third day without anaphylaxis, again responding to prednisone and hydroxyzine. Results: We believed her cutaneous reactions were not IgE-mediated; rather they were T-cell mediated, occurring within a few days of the initiation of two beta blockers. There are no validated skin testing or in vitro serum testing assays available for proving beta-blocker sensitivity. We empirically suggested the patient start a low dose beta-blocker under cover of oral cyclosporine in an attempt to abrogate the cutaneous reaction. With the reinitiation of carvedilol the patient developed no rash, dermal pruritus, or angioedema. After two months the cyclosporine dose was slowly tapered, the carvedilol dose was escalated, and such was well tolerated. After 6 months, we recommended that the patient discontinue the low dose cyclosporine (25 mg twice daily) while continuing the carvedilol (12.5 mg twice daily), but the patient relocated to Europe prior to dose changes. Conclusions: Cyclosporine has been used off-label for the treatment of various inflammatory and autoimmune skin conditions. This is a prime example of successful use of cyclosporine to prevent a drug-induced skin reaction. Further studies need to be performed to evaluate the efficacy of cyclosporine in T cell-mediated, drug-induced allergic dermatoses, wherein the use of such drug is deemed necessary. T. Kelbel * , F. Ishmael, Hershey, PA. Introduction: The diagnosis of T-cell mediated drug hypersensitivity reactions is challenging due to lack of sensitive and specific assays. We describe a case of delayed hypersensitivity reaction to docetaxel and use of an in vitro assay using T-cell gene expression response to characterize the reaction and preemptively test navelbine as a safe, alternative chemotherapy treatment. Patient History: A 59 year old woman with stage 3 breast cancer developed whole body erythematous rash shortly followed by desquamation about 24 hours after cyclophosphamide and docetaxel treatment. It was not clear whether the reaction was caused by cyclophosphamide or docetaxel. Methods: An in vitro Tcell assay was used to quantify gene expression changes in a panel of cytokines and inflammatory mediators (IL-2, -4, -5, -6, -8, -10, -13, -17a, GM-CSF, TNFα, CCL2, IFN-γ, Fas ligand) when exposed to docetaxel and cyclophosphamide. Additionally, her oncologist requested navelbine be preemptively tested as it would be indicated as her next chemotherapeutic option. Results: In vitro testing showed significant elevation of T-cell response of IL-6 and CCL2 to docetaxel compared to cyclophosphamide and navelbine ( Figure) . These results suggest that the reaction was a type IVa delayed hypersensitivity reaction to docetaxel. The patient was successfully treated with navelbine and cyclophosphamide and is currently in remission. Conclusion: In vitro T-cell response testing may be used to characterize docetaxel allergy while also predicting the absence of a type IVa reaction to navelbine. This is a novel approach to chemotherapeutic drug reactions with significant clinical application potential. Nevertheless, more research is needed before mainstream application. P. Oza * , Ann Arbor, MI. Introduction: Drug induced hypersensitivity reaction to amlodipine is rare. We present a 59 year old woman who presented with a 3 month history of a cutaneous manifestation drug induced hypersensitivity which started 2 days after she was re-started on Amlodipine. Methods: Evaluation, diagnosis, and treatment of a patient with serum sickness secondary to amlodipine. Results: A 59 year old woman presented with a 3-month history of severe rash which started 2 days after being placed on Amlodipine for uncontrolled hypertension. The patient recalls taking the drug before, but did not know the duration or why she stopped the therapy. Her rash was located primarily on her palms and soles. It was painful, pruritic, showed significant desquamation and was associated with a constant burning sensation. She also had swelling of her legs. Amlodipine was discontinued after 2 weeks, but the rash persisted. She was treated with a Medrol dose pack which improved her symptoms, but the lesions recurred despite follow on therapy with Clobetasol cream BID, Vaseline, Vitamin E, and Benadryl. Her pain was debilitating and prevented her from walking. Dermatology obtained a punch biopsy of her hands and feet revealed eczematous dermatitis, and was noted to have mild largely perivascular dermal lymphoid infiltrate. She was prescribed modified Goeckerman regimen which was not helpful. Upon presenting to our clinic, her rash was clearly demarcated by erythematous marginal lines present on both palms and soles (see image). Her ESR was noted to be elevated at 30. She was started on a prednisone taper. At her 2 week follow up visit, she showed resolution of the cracking on the palms and burning sensation, with presence of mild residual erythema. Conclusion: There have been only 3 case reports suggesting a cutaneous reaction to amlodipine use. The cutaneous manifestations of serum sickness are variable. Prominent skin changes at the lateral aspect of the feet and hands, especially at the junction of the sole and side of the foot and at the junction of palm and dorsal skin surface has been noted to be unique to serum sickness. Given that amlodipine is lipid-soluble, it can take months for the body to clear, which explains the persistent nature of the rash. Conclusion This case demonstrates Amlodipine induced drug hypersensitivity can present as an isolated cutaneous manifestation that is responsive to treatment with oral steroid. S. Spriet * , T. Banks, T. Love, Bethesda, MD. Introduction: Drug reaction with eosinophilia and systemic symptoms (DRESS) is a rare, drug-induced reaction characterized by rash, fever, lymphadenopathy, elevated liver enzymes, and leukocytosis with eosinophilia and has a long latency period between exposure and disease onset. We describe a case of recurrent DRESS triggered by a structurally unrelated drug. Case Presentation: A 54-year-old female with Neurofibromatosis type I and seizure disorder presented to her primary physician with a diffuse morbilliform eruption and facial edema approximately 4 weeks after transitioning from levetiracetam to lamotrigine. As a result, lamotrigine was discontinued; however, the patient was soon admitted due to persistent cutaneous symptoms and new fever, abdominal pain and dyspnea. Laboratory studies were significant for a leukocytosis of 35,000 cells/mcL, an absolute eosiniphilia count of 7500 cells/mcL, elevated liver enzymes and an increase in serum creatinine from a baseline of 0.7 to 2.7 mg/dL. Bilateral pulmonary infiltrates were noted on chest radiography. There was no mucosal involvement. Intravenous corticosteroids were initiated and the patient's symptoms and laboratory values steadily improved. Following discharge, the patient continued on a planned oral steroid taper. Three weeks later she was started on a ten day course of clindamycin (1200mg/day) for treatment of a newly diagnosed cellulitis. This process quickly resolved, but on day 10 of the antibiotic course she developed a recurrence of her morbilliform rash, evolving to significant erythroderma that resolved with an increase in the daily dose of prednisone from 50 to 60mg. Due to the clinical course and temporal relation to clindamycin, the patient was diagnosed with recurrent DRESS triggered by the antibiotic. Discussion: Antibiotics are the most commonly implicated drugs in cases of recurrent DRESS however, the mechanism by which these or other structurally unrelated agents trigger the abnormal immune response remains unclear. As in our case, recurrent reactions tend to be limited to cutaneous findings. Providers should be aware of this potential complication in patients with a history of DRESS and recognize the need for prompt treatment. Bacitracin is a polypeptide antibiotic that was approved by the FDA in 1948. Topical bacitracin ointment has traditionally been used to prevent skin infections. More recently prophylactic bacitracin irrigations are being used during surgical procedures. Seven cases of intraoperative anaphylaxis after exposure to bacitracin irrigations have been previously reported. Here we present the cases of two patients who developed hypersensitivity reactions after receiving prophylactic bacitracin irrigations. The first case is a 47 year-old female with a history of breast cancer status post mastectomy and prior patch testing that was positive for bacitracin who presented for breast revision. Upon starting the procedure, lidocaine 1% with epinephrine was employed for local anesthesia and the surgical wound was irrigated with a bacitracin solution. A few minutes after receiving these medications, she developed generalized erythema and hypotension. Intravenous hydrocortisone, diphenhydramine, and famotidine were given with improvement in her blood pressure. Full recovery ensued. Laboratory tests done later that day were all normal, except for an elevated serum tryptase at 77.9 mg/L (normal <11 mg/L). Subsequent skin testing for lidocaine and latex was negative. The second case is a 51 year-old female with a history of breast cancer status post mastectomy who also presented for a breast revision. She was given cefazolin before the procedure, prepped with chlorhexidine and, as in the prior case, the surgical wound was irrigated with a bacitracin solution. No immediate complications were noted. One day after the procedure she developed a pruritic erythematous papular rash over the left breast that later spread to her back and abdomen. The rash resolved after completing a short course of oral prednisone. Bacitracin patch testing done in our clinic was positive. In addition, skin testing for penicillin and chlorhexidine was negative. In summary, these are two very interesting cases of patients who developed hypersensitivity reactions after intraoperative exposure to a bacitracin solution. This problem is particularly relevant since prophylactic bacitracin irrigations of clean surgical wounds are becoming more common. C. Collins *1 , J. Sherr 2 , A. Liu 2 , 1. Cupertino, CA; 2. Palo Alto, CA. DRESS (drug reaction with eosinophilia and systemic symptoms) is a serious and sometimes fatal drug reaction that is difficult to diagnose. Mexiletine has been reported as a cause of DRESS in case reports in the adult literature. Here we report the first case of drug reaction with eosinophilia and systemic symptoms (DRESS) in a pediatric patient caused by the antiarrhythmic drug mexiletine. Our patient developed DRESS on mexiletine started for non-sustained ventricular tachycardia associated with nonischemic idiopathic cardiomyopathy. Her symptoms included fever, rash and facial swelling, lymphadenopathy, and peripheral eosinophilia approximately 6 weeks after starting mexilitene. Though mexilitene was initially discontinued with improvement in reaction symptoms, it was restarted when the patient had multiple PVCs and there was concern for the subsequent development of ventricular tachycardia, for which she did not have good long-term alternatives. This case is unusual and significant because a re-exposure was associated with syndrome recurrence, confirming the culprit medication. Perioperative anaphylaxis is often a difficult diagnostic dilemma as multiple medications are administered in a short timeframe. Considering all agents in this setting is prudent. Anaphylaxis due to corticosteroids is rare with only about 100 case reports available. Hydrocortisone, prednisolone, and methylprednisolone are the formulations most often implicated in anaphylactic reactions. We present a case of near-fatal anaphylaxis attributed to methylprednisolone succinate. A 45-year-old female presented for ophthalmological surgery requiring general anesthesia. Within 30 minutes, the patient suffered severe hypotension and cardiac arrest with pulseless electrical activity. She was resuscitated and transferred to the ICU. The Allergy/Immunology service was asked to confirm anaphylaxis and identify the culprit. Our institution has recently adopted a new electronic medical record system (EMR). The system allows recording of operative events which are presented in graphical format including vital signs and medication administration in the context of time. This format proved helpful in identifying the causative agent of anaphylaxis in this case. According to the operative record, the patient entered the OR in stable condition. Induction of anesthesia included propofol, rocuronium, and fentanyl. She also received ocular lidocaine. Approximately 30 minutes later, a second dose of rocuronium was administered with 500mg of IV methylprednisolone. Vital signs were stable prior to dosing; however, within minutes, severe hypotension and cardiac arrest ensued. The patient received epinephrine and CPR with return of spontaneous circulation. There were no reports of rash or flushing. Using information from the EMR graph, skin testing was planned for rocuronium, cisatracurium, fentanyl, and methylprednisolone. SPT and intradermal testing to rocuronium, cisatracurium, and fentanyl were negative. SPT to methylprednisolone was positive with wheal/flare of 10/35mm. Positive and negative controls had wheal/flare of 10/30mm and 0/5mm, respectively. This case illustrates the need to consider corticosteroids as a cause of anaphylaxis. The EMR and graphical record are useful tools in recognizing the timing of anaphylaxis and guided drug testing. The EMR in combination with positive and negative SPT results helped in making a diagnosis of methylprednisolone anaphylaxis. Background: Anaphylaxis is a severe and potentially life-threatening reaction caused by an IgE-mediated systemic response to drugs, foods, and other allergens. Anaphylaxis associated with laboratory animal exposure is a recognized but rare occurrence. The development of atopic symptoms such as rhinoconjunctivitis and asthma is common among those who work with laboratory animals, estimated to affect 11-44% in close contact with rats or mice. However, only a few cases of true anaphylaxis have been reported. Objective: To report a case of anaphylaxis which occurred after rat bite in a laboratory worker with history of atopic disease exacerbated by rat exposure. We will review this case and published literature for diagnosis and management guide-lines. Materials and Methods: We will review the medical record of this patient, including results of laboratory and RAST testing. We conducted a MeSH search for "anaphylaxis", "rat", "mouse", "murine", and "bite". Results: Review of literature revealed 7 previously reported cases of severe allergic reaction after rat or mouse bites. One case was an anaphylactoid reaction in a 9-year-old that was bitten by a pet mouse. 6 other cases of true anaphylaxis involved laboratory workers. 5 of the 7 previously reported reactions occurred in patients with atopy exacerbated by murine exposure. Our patient was evaluated in the ER for an episode of anaphylaxis that occurred minutes after sustaining a rat bite to the finger. He was treated with epinephrine, prednisone, and antihistamines with prompt recovery and referred to our clinic for follow up. We used RAST testing to assess for sensitization to laboratory animals, and he was found to have strongly positive IgE to rat and mouse urine and epithelium (rat urine IgE>100). Recommendations were made for avoidance of laboratory animals, and he was provided with an epinephrine autoinjector. Conclusion: Murine anaphylaxis is an unusual but important entity to recognize in the workplace. Laboratory workers who develop allergic sensitization to animals as evidenced by exacerbation of asthma and rhinoconjunctivitis should be aware of the risks of development of serious reactions, and career counseling may be indicated. Murine specific immunotherapy can also be considered. The genetic and molecular bases of murine anaphylaxis are unknown and require further study. Background: The stability of short ragweed allergens in immunotherapy vaccines can be compromised by mixing with other extracts, particularly those containing active hydrolytic enzymes. Delivery of inaccurate or inconsistent allergen levels can reduce the effectiveness of patient treatment regimens using these mixtures. Objectives: To confirm the compatibilities of standardized short ragweed extracts in mixtures with a variety of other phenolated, glycerinated, high-protease (mold, cockroach) and low-protease (tree, grass, cat, dog epithelia, dust mite) extracts at concentrations targeted for maintenance immunotherapy administrations. Methods: Two-part and three-part mixtures containing short ragweed and other glycerinated extract concentrates were analyzed by Amb a 1 radial immunodiffusion assay, the method currently used for short ragweed extract standardization, after storage for up to 12 months at 2-8°C or up to 6 months at 20-25°C. Results: Short ragweed extract controls and all mixtures displayed favorable Amb a 1 recoveries during storage at either temperature. Control sample recoveries ranged from 90-107 (2-part) and 49-72 (3part) Amb a 1 U/mL, and mixtures retained 85-117% (2-part) and 70-116% (3-part) of control values in these analyses. There was no evidence of progressive changes in short ragweed extract potency in the stored samples. Conclusions: Diverse combinations of short ragweed and other glycerinated extracts frequently used for immunotherapy displayed very stable ragweed allergen levels under all conditions examined. These data support refrigerated storage of these and related extract mixtures for up to 12 months in clinical settings. Ambient temperatures (20-25°C) were evaluated as an accelerated (worst-case) experimental condition, and are not recommended for storage of actual patient immunotherapy formulas. Background: Preparation of optimal extract mixtures for allergen immunotherapy can be restricted by changing regulations or limits for reimbursement. Stabilization of allergen combinations known to be incompatible under conventional storage conditions, such as pollens mixed with proteaserich fungi or insects, can provide viable alternatives to help allergists administer the desired immunotherapy formulations to their patients. Objectives: To examine the compatibilities of standardized Meadow fescue and Timothy grass pollen extracts in mixtures containing fungal or insect extracts at elevated glycerin concentrations after storage for up to 12 months at refrigerator (2-8°C) or freezer (-15 to -20°C) temperatures. Methods: Meadow fescue and Timothy extracts were combined with individual fungal (Alternaria, Aspergillus, Penicillium) or insect (American cockroach, German cockroach, fire ant) extracts at glycerin concentrations ranging from 25-50%, in 5% increments. Extract levels corresponded to those producing effective maintenance doses for subcutaneous immunotherapy. Grass extract potencies were determined by IgE ELISA inhibition assay, the same method used to standardize these products. Results: Grass allergens in mixtures with fungal or insect extracts were stabilized considerably by storage in a conventional freezer. Several mixtures at 25-30% glycerin were partially frozen, but those at 35-50% glycerin remained in liquid phase. All combinations exhibited significant improvements in grass allergen potency relative to identical mixtures maintained at 2-8°C (up to 10fold for Meadow fescue; up to 16-fold for Timothy). Recoveries for most mixtures at 25% glycerin in freezer exceeded those at 50% glycerin in refrigerator. Conclusions: Storage of glycerinated extract mixtures at freezer temperatures stabilizes labile allergens, supporting formulation of specific product combinations that must currently be separated into different treatment vial sets. Background: Aeroallergen surveys help clinicians select appropriate allergens for skin testing and immunotherapy.Knowing local pollen and spore patterns might also help optimize access to care and scheduling for patients. Objectives: To evaluate patterns of pollen and spore dispersal in Louisville, Kentucky and correlate these with the number of new appointments scheduled with an Allergist-Immunologist and with the number of acute visits for pediatric asthma. Methods: A National Allergy Bureau certified counter in Louisville, Kentucky performed daily pollen and spore counts.The mean daily pollen/spore count was determined and graphs were generated indicating the onset, peak, and duration of total tree, grass, and weed pollen and mold spores.These graphs were compared to the number of monthly new office visits in a large Allergy-Immunology practice.In addition, the pollen counts were compared to the monthly emergency room visits and admissions for asthma in a large children's hospital. Results: Daily pollen/spore counts were available for 7 of 10 years from 2003-2013. Tree pollen was present from mid-February through mid-June with a bimodal peak; the first in mid-March dominated by juniper and the second in mid-May dominated by oak. Grass pollen was present from April through August with a peak in mid-May. Weed pollen increased in mid-August with a peak in early September and resolution by late-October. Mold spores were present year round, but began rising around the first week of April, with maximum levels occurring from June 01 through October 01, and decreasing to baseline winter levels in early December. New allergy visits showed a bimodal peak with the first occurring in early May and the second in late September. These peaks occurred approximately 4 weeks later than peak pollen and the adjusted total monthly pollen curve demonstrated significant correlation with new office visits (R 2 =0.725). The pollen curve also correlated with total monthly asthma visits from March through September (R 2 =0.77), but asthma visits and pollen count did not correlate when including the winter months in the data set (R 2 =0.03). Conclusions: Acute asthma visits correlate with the pollen count during the pollen season and demand for new office Allergy-Immunology visits correlates with peak pollen counts with a lag time of about 4 weeks. Rationale: Hayfever symptoms directly correlate with atmospheric pollen concentrations. Climatic and environmental factors have the ability to influence local botany and aerobiology. National Allergy Bureau pollen count stations determine the presence of local, clinically relevant allergens and track patterns of prevalence. This data is extremely valuable to treating allergists as knowledge regarding yearly and seasonal fluctuations in pollen counts is important in the managing patients with allergic rhino-conjunctivitis, in order to provide appropriate treatment regimens. Methods: Atmospheric sampling for aeroallergens in Atlanta, GA was preformed over a nine-year interval according to National Allergy Bureau standards using Rotorod sampler. Samples were collected each morning by trained observers and various pollens are counted. Atmospheric concentrations are determined by calculation from the raw counts. The pollen counting station was moved a distance of 2.2 miles in 2010. The staff is certified through the American Academy of Allergy, Asthma and Immunology Aeroallergen Network. Results: The southeast has an intense spring pollen season with very high tree pollen counts. Late summer-fall pollen season in past years is less intense and characterized by a ragweed bloom. However, through 2009-2013, there were very high levels of Cedar Elm during August and September. The pollen counts were consistently within the moderate range as determined by NAB criteria as greater than 15 grains per cubic meter. Peak counts ranged between 39.1-475.2 grains/cubic meter and 4-day average ranged between 23.5-206.2 grains/cubic meter. Notable, was the increasing duration of the season and days spent within the moderate pollen range. Conclusions: Cedar Elm has not been a dominant local aeroallergen in Atlanta, Georgia. It appears that due to a variety of climatic and environmental factors over the last several years, the prevalence of Cedar Elm pollen in metro-Atlanta has increased. The change of positioning of the pollen collection device may also be a factor for these extremely high counts, nevertheless Cedar Elm is an important aeroallergen and should be considered in patients having predominant summer-fall symptoms, especially if they fail to respond to Ragweed testing or immunotherapy which presents during the same time of year. Introduction: Medical adhesive bandages frequently cause irritant contact dermatitis, occasionally allergic contact dermatitis. Systemic hypersensitivity reactions from the adhesive bandages are exceptionally rare. We report alginate, a component of compression bandages used after hemodialysis (HD), as causing anaphylaxis. Case: A 31-year-old woman with end stage renal disease due to prolong hypertension, received HD therapy via arteriovenous fistula for 8 years, presented with 5-months of recurrent generalized pruritus urticarial eruption, along with shortness of breath immediately after hemodialysis sessions. She had no history of angioedema; and no history of chemical, medication or food allergies. Skin prick testing to hydrocolloid gel from TipStop® (Gambo, Hechingen, Germany) bandage was positive and negative to medications administered at the time of HD including intravenous iron, erythropoietin and paricalcitol. Total IgE, latex IgE and tryptase levels were unremarkable. The patient was advised to avoid TipStop® bandage and became symptoms free for 3-month follow up. Discussion: TipStop® is one of the modern compression bandage that claims to be hypoallergenic. The Bandage has hydrocolloid gel in the center made from alginate which is designed to stop bleeding after vascular punctures and to protect the fistula. Alginate, an extract from brown seaweed, is used extensively in medical devices and products such as dental impressions, prosthetics, soft tissue dermal fillers, dressings and compression bandages. It is also used in food industry, for thickening soups and jellies. This is the first case of anaphylaxis to bandages. The more severe reaction in our case might be caused by direct contact of alginate with vascular system. Conclusion: The allergenic potential of modern wound dressings should not be underestimated. Background: Asthma exacerbations triggers include environmental factors. Objective: The objective of this study was to investigate the relationship between Emergency Department visits for asthma and pollen counts. Methods: The number of emergency department visits for Asthma between the years 2008-2013 was recorded at John Muir Hospital in Walnut Creek, California. Pollen counts were collected in Pleasanton, California during the same time period. Results: The number of emergency department (ED) visits for Asthma varied throughout the year with a peak number of visits occurring in May. We found a significant correlation between the number of Asthma ED visits and: 1. The monthly averaged total pollen counts (rho=0.629, p<0.05) and 2. The monthly averaged tree pollen counts (rho=0.678, p<0.05). The daily ED visits for Asthma significantly correlated with: 1. The total pollen counts (rho=0.212, p<0.0001), 2. The grass pollen counts (rho=0.192, p<0.0001) and 3. The tree pollen counts (rho=0.201, p<0.0001). There was a significant difference in the number of daily visits between days with high grass pollen counts vs. low grass pollen counts (Mann Whitney. p<0.0005. High grass pollen days: Mean=2.024 visits per day. Low grass pollen days: Mean=1.302 visits per day). A significant difference in the number of daily visits was also observed when comparing days with high tree pollen counts vs. low tree pollen counts (Mann Whitney. p<0.001. High tree pollen days: Mean=1.894 visits per day. Low tree pollen days: Mean=1.312 visits per day). Conclusions: ED Asthma visits in this study were associated with tree pollen counts and with grass pollen counts. Monthly averages of the different pollen types and ED Asthma visits between the years 2008-2013 with SEM. Pollen data presented as pollen grains per cubic meter. Asthma visits data presented as the monthly averaged number of visits. Introduction: In the US, the standardized extracts, grass pollen, dust mite, short ragweed, cat hair/pelt, and venoms have expiration dating determined by formal manufacturer studies and approved by the FDA. Non-standardized extracts have expiration dating specified by the FDA. 50% glycerin extracts are dated for 6 years from extraction, but only 3 years from shipping, and nonglycerin extracts are dated for 3 years and 1.5 years from shipping. Little evidence is available to support this dating, so the purpose of this study is to evaluate various extracts produced over the last 10 years for protein and allergen content. Methods: Allergen extract concentrates were analyzed for protein diversity by SDS-PAGE. Potency of some extracts was determined by IgE binding and major allergen assays such as Bet v 1 in Birch, Pla l 1 in English plantain, Ara h 1,2,6 in peanut and tropomyosin in shrimp. The commercial extract lots were manufactured over a 10 year period and were stored in their final container vials at 2-8C. Results: SDS-PAGE protein patterns generally maintain consistent patterns for the mandated expiration dating. Most aqueous extracts began to show changes after the 3 year expiration and most glycerin extracts showed little change over 6 years. Remarkably, some extracts maintain consistent patterns for up to 10 years. Potency assays helped support the gel data. Conclusion: The characterization methods used in this study provide evidence for the expiration dating for many non-standardized allergen extracts. The stability enhancing ability of glycerin has been confirmed. T.J. Walker * , L.M. Steacy, B. Hobsbawn, A.K. Ellis, Kingston, ON, Canada. Background: Certainty of continual Rotorod® impact sampler revolutions per minute(RPM) is unknown during normal operation using accepted traditional design and RPM calibration techniques; only during RPM calibration can one be definitive. As EEU pollen concentration levels are monitored and\or adjusted based on accurate sampling, monitored constant RPM values are preferred. Methods: Custom programmable microcontroller boards, optical encoder technology, secure digital(SD) storage, wireless connectivity, and touch screen control are combined to provide real time monitoring, alerts, and data storage. Pulse width modulation(PWM) using the proportional-integral-derivative controller(PID) and encoder feedback ensure accurate motor control. The 2400 RPM speed is calculated and recorded per second and stored along with device name, date, time, and user stamp on the onboard storage or wirelessly to a separate location. A standard 30 second spin cycle was initiated multiple times over a 15 minute period while simultaneously taking RPM measurements with a handheld National Institute of Science and Technology(NIST) certified optical tachometer. Voltage was monitored until the rotorod system was unable to sustain the minimum RPM and noted. All data was stored and then compared in our central database. Results: The RPM comparison speeds captured by the new system averaged 2380.90 RPM, st dev 35.86. Readings from the handheld tachometer averaged 2394.52 RPM, st dev 9.65. The minimum voltage causing RPM under tolerance was 11.85 volts. The new design allows for continual, noncontact, real time monitoring and storage of data from multiple devices simultaneously, therefore, lessoning labor time requirements. Conclusions: The redesigned rotorod particle sampler will allow for real-time verification feedback and alerts for all particle sampling in the EEU. This advancement assures that all particle sampling using rotorod impact sampling is accurate for the entire duration of all duty cycles. Qualification/ validation of these devices addresses any mitigations deemed necessary to meet regulatory guidelines. Background: Orchard workers are highly exposed to several allergic agents, especially peach pollen and other Rosaceae family pollens. However, no occupational allergy in this branch has been described before. Methods: First, skin tests, serum-specific IgE tests with home-made extracts of peach pollen and other four Rosaceae family pollens (apricot pollen, cherry pollen, apple pollen and pear pollen) of the working environment were executed. Furthermore, conjunctival challenge with peach pollen was performed, western immunoblots with these five Rosaceae family pollens were performed. In addition, ELISA inhibition and western blotting and inhibition experiments among five Rosaceae family pollens were performed. Result: Skin prick tests with five Rosaceae pollens extracts were positive in patient. Specific IgE against three pollens (peach pollen, apricot pollen and cherry pollen) could be demonstrated in the patient by ELISA. Cross reactivity studies by ELISA inhibition performed with the patient's sera, when peach pollen used as solid phase, IgE binding to peach pollen could be inhibited by precubation the other four pollens with inhibition rate ranging from 45% to 87%, the strongest inhibitor was apricot pollen extract. Sera from patient showed clear IgE binding to 20kD,60kD,80kD proteins in peach pollen extract, similarly the patient's sera recognized apricot pollen proteins of 20KD,55kD,80kD, the sera showed weak IgE binding to 20KD,80KD component of cherry pollen, and the IgE reactivity protein in apple pollen appeared to be 80kD and 110 kD. The 20KD IgE binding component in patient's serum could be demonstrated with peach pollen, apricot pollen and cherry pollen. The IgE binding to the 20kD protein in peach pollen extract was completely inhibited with apricot pollen (10mg/ml) as inhibitor. When cherry, apple, and pear pollen were used as inhibitors, 20kD band was partly inhibited. Conclusion: We conclude that peach pollen is a potential cause of IgE mediated occupational respiratory disease, extensive cross reactivity among Rosaceae pollens. An increasing number of patients are needed to confirm these interesting preliminary finding, and epidemiologic studies are necessary to assess the importance of this aeroallergen among exposed orchard workers. UNIQUE ALLERGY AND ASTHMA SMARTPHONE APP. S. Kagen * , Appleton, WI. Smartphone applications are now playing a new and critical role in monitoring the success or failure of allergy and asthma treatments, especially as new healthcare reforms reward successful patient outcomes and performances. Monitoring allergy patients has been difficult, but a new and unique Smartphone software application (KagenAir ® ) enables consumers to simultaneously monitor their allergy and asthma symptoms and discover local environmental factors that affect how they feel using patent pending methodologies and computer generated animated maps and graphics. The KagenAir App is unique in that it measures symptoms scores and also directly connects consumers with uncontrolled allergy and asthma symptoms to Board Certified Allergy-Asthma-Immunology Specialists within their communities using both cell phone and video-chat technologies. Beta testing on allergy and asthma patients established the KagenAir App to be easy to understand, simple to use and helpful in connecting consumers with local allergy specialists. Background: Though exposure to pets is a known trigger of allergic disease, it has been shown that such exposures, particularly at a young age, can be immunoprotective. Early dog exposure especially has been associated with decreased risk of wheezing and atopic dermatitis. Methods: Data from the Cincinnati Childhood Allergy and Air Pollution Study (CCAAPS) provided cat and dog allergen levels contained within dust samples collected from the homes of infants born between the years of 2001 and 2003 with at least one atopic parent. Cytokine levels were measured from blood samples obtained when CCAAPS study participants were 11-12 years of age. IRB approval and informed consent was obtained from all research subjects. The study data from the first 40 participants with available serum samples were included in this study, and levels of cytokines were measured via a Luminex® multiplex cytokine assay. High allergen level was defined as a level at or above the calculated median. High cytokine level was also defined as a level at or above the calculated median. Differences in the proportions of high and low allergen levels with high and low cytokine levels were tested by Chi-square. Results: Although the differences in cytokine levels did not reach statistical significance, impor-tant trends were noted. Study participants with early life exposure to high levels of dog allergen had a nearly 50% reduction in levels of the following cytokines compared to those with exposure to low levels of dog allergen: G-CSF, IL-10, MCP-3, TGFalpha, IL-9, and IL-3. No cytokine was elevated in participants with exposure to high levels of dog allergen compared to the low dog allergen level group. In contrast, study participants with early exposure to high levels of cat allergen had significantly higher levels of the following cytokine compared to those with exposure to low levels of cat allergen: IL-12p70, G-CSF, Mip-1alpha, IL-9, and TNFbeta. Only VEGF was substantially elevated in participants with exposure to low levels of cat allergen. Conclusions: The observed trends support that early exposure to high levels of dog allergen is immunoprotective and associated with lower serum levels of cytokines in later childhood. Notably, a similar relationship was not found with high cat allergen exposure at a young age. In contrast, early exposure to high levels of cat allergen was associated with higher cytokine levels. M.Z. Braunstein * , R. Joks, Brooklyn, NY. Background: Obesity has been linked to asthma exacerbations, total serum IgE, and severity of atopic dermatitis. The correlation of obesity on allergy testing responsiveness has not been well studied. Objective: We sought to investigate the relationship of BMI to allergen skin prick tests and serum specific allergen IgE levels of common aeroallergens and foods. Methods: We reviewed adult patient charts (n=288) who presented to the allergy and immunology clinic from 1998-2013. Data from patients with physician diagnosed allergic rhinoconjunctivitis or asthma who underwent routine epicutaneous skin prick testing (SPT) for common environmental allergens (n=124) or who had allergen specific IgE levels (FEIA) to common environmental and food allergens (n=92) were included for analysis. Spearman coefficients were generated to determine an association of BMI with the number of positive SPT, the mean wheal diameter, and the maximum specific IgE class. Results: BMI significantly correlated with the number of positive SPT (p=0.01) and inversely with the mean wheal diameter (p=0.05). There was no significant correlation with the maximum specific IgE class. Conclusions: Obesity, as measured by BMI, is associated with increased specific cutaneous allergic responses. However, the cutaneous response to allergen decreases with increasing BMI, suggesting an attenuated response to allergic mediators released during immediate hypersensitivity reactions with increasing BMI. Background: Cancer is an important cause of health morbidity in adults. One in four deaths are due to cancer (ACS, 2014). There is little published literature of serum IgE and eosinophil level associations with cancer on a national level. Objective: To examine the association between serum IgE, eosinophil % and the overall vs. type-specific prevalence of cancer. Methods: Data from a nationwide survey conducted by the Centers for Disease Control and Prevention, National Health and Nutrition Examination Survey (NHANES 2005 (NHANES -2006 was used. Bivariate analyses with chi-square tests, logistic regression and stratified models assessed the relationship between serum IgE and eosinophil %) and the overall prevalence of cancer (defined as ever having been diagnosed by a physician with cancer of any kind) versus the type-specific prevalence of cancer. Results: A total of 4,893 adults constituted our study population. After controlling for age, gender, ethnicity, education and income, smoking status and duration of cancer diagnosis, subjects with high serum IgE had a negative odds of overall cancer (OR = 0.83, 0.64-1.09), as did subjects with higher eosinophil levels (OR = 0.90, 0.82-0.99). Interaction models demonstrated the significant protective effect of cancer for IgE for males (OR= 0.85, and eosinophil levels in females (OR= 0.73, 0.54-0.98). Finally, IgE levels had a negative odds of lung (OR= 0.28, 0.15-0.52) and cervical cancers (OR= 0.84, 0.61-0.16). High eosinophil levels also had negative odds of lung (OR=0.39, 0.15-1.00) and cervical cancers (OR=0.57, 0.41-0.78). Conclusion: This study shows that atopy and eosinophilia were associated with a decreased prevalence of overall cancer after covariate adjustment, using a large, nationally representative dataset. This suggests the need for continued allergy surveillance and longitudinal studies of the possible protective role of serum IgE and eosinophil levels in lung and cervical cancers. Introduction: Allergy to metals and acrylates in bone cement have been implicated in joint implant failures. Over 900,000 hip and knee replacement surgeries are performed annually in the US. The purpose of this study was to evaluate contact hypersensitivity to metals and acrylates in patients undergoing joint replacement. Methods: Sixty-five subjects were referred for evaluation of possible allergy to metal between 2012-2014. Subjects underwent either pre-operative (N=46) or post-operative (N=19) patch testing to a standard panel of metal salts (nickel, cobalt, chromium, palladium, vanadium, and zirconium) and methyl and ethyl acrylate. Patients and referring providers were contacted for information regarding component materials in the prostheses and postsurgical outcomes. Results: Of 65 patients, 46 with a self-reported history of metal allergy were tested pre-operatively (PRE) and 19 patients were referred postoperatively (POST) for implant failure. Thirty-five (76%) of the PRE group had a positive patch test; all these patients had an alternative material (eg zirconium, polyethylene, ceramic, or titanium) implanted. Fourteen of POST patients had positive patch tests (74%); 8/14 (57%), however, gave no prior metal allergy history. Nickel was the most common offending antigen in both groups with 54% of PRE and 32% of POST patients reacting to nickel. Rare metals such as palladium caused several reactions with 8 PRE and 4 POST positive patch tests. Two PRE and 3 POST patients, all female, reacted to ethyl acrylate as well. Conclusion: A history of metal allergy and/or contact hypersensitivity confirmed by patch testing may influence choice of implant material. In addition to infection and biomechanical failure, metal allergy should be considered as a potential cause of prosthetic joint failure. Introduction: A 48 year old male presents with a 20 year history of chronic urticaria that has been worsening in severity for the past 4 years. He reports a long-standing pruritic urticaria that comes and goes on his arms, legs, abdomen, and back. For the past 4 years he has had more painful lesions occurring over a greater area and with increased frequency, almost daily. The pain and itch are relieved by oral steroids and antihistamines, but he has been requiring greater doses of short-acting antihistamines recently. On review of systems, he also reports nasal congestion, cough, abdominal pain, BRBPR due to hemorrhoids, joint aches and swelling. Social history revealed a 25 pack-year smoking history, heavy alcohol consumption, and previous incarceration for ten years. Physical examination revealed raised, erythematous, circumscribed lesions that are not blanchable on his upper and lower extremities, abdomen, and back. Results: Laboratory evaluation revealed mild anemia Hb13.1g/dL, Hct 39.6%. IgE 28 kU/L. CMP unremarkable. Absent CH50 and C4. Low C2 (0.9mg/dL) and C3 (36mg/dL). Rheumatoid factor positive. C1q, ANA, ANCA, ESR (2mm/hr), anti-Smith, anti-dsDNA and anti-RNP antibodies were normal. SPEP unremarkable. Quantiferon Gold negative. HIV negative. Hepatitis C antibody reactive, HCV Quantitative 1,160,000 IU/mL and HCV Quantitative Log 6.06. Hepatitis C genotype 1b. Biopsy was consistent with vasculitis. Cryoglobulins positive. The patient is scheduled to start anti-viral therapy. Discussion: Cryoglobulinemia is a systemic inflammatory syndrome that involves small-tomedium vessel vasculitis due to immune complexes. Clinical presentations of cryoglobulinemia vary widely. Cryoglobulinemia has different underlying etiologies, and is associated with up to 65% of patients with hepatitis C virus. Cryoglobulinemia can cause a triad of palpable purpura, arthralgia, and myalgia when viral associated, while cryoglobulinemia due to monoclonal immunoglobulins or hematological malignancies is generally associated with hyperviscosity. Cryoglobulinemia is diagnosed by the demonstration of serum cryoglobulins in association with characteristic clinical signs and symptoms. Prognosis is generally dependent on the degree of resolution of the underlying condition. This case marks the importance of an infectious work-up and vasculitic evaluation in a patient with non-remitting urticarial lesions. O.H. Al Jiffri * , Jeddah, Saudi Arabia. Background: Weight loss studies were conducted in children without asthma have demonstrated a reduction in systemic inflammation. However, the impact of weight loss in the obese paediatric population with asthma has not been investigated. Objective: To measure the effects of weight loss on markers of systemic inflammation in obese children with bronchial asthma. Methods: Eighty obese children with bronchial asthma (42 boys, and 38 girls) with mean age 13.86± 3.21 years were divided into two equal groups. The training group received diet regimen, exercise training in addition to the medical treatment for two months, where the control group received the medical treatment only. Results: There was a 17.5%, 15.5%, 22.4%, 14.1% and 15.9% reduction in mean values of tumor necrosis factor-alpha (TNF-alpha), interleukin-6 (IL-6), interleukin-8 (IL-8), Leptin and body mass index (BMI) respectively and 38.7% increase in mean values of adiponectin in the training group. While, there was a 0.7%, 9.0%, 2.8%, 1.6% and 1.2% increase in mean values of TNF-alpha, IL-6, IL-8, Leptin and BMI respectively and 3.9% decrease in mean values of adiponectin in the control group. The mean values of TNF-alpha, IL-6, IL-8, Leptin and BMI was decreased and adiponectin was increased significantly in the training group, however the results of the control group were not significant. Also, there were significant differences between both groups at the end of the study. Conclusion: Weight loss improves markers of systemic inflammation in obese Saudi children with bronchial asthma. Key words: obesity, asthma, weight loss, cytokines. Mean value and significance of TNF-α, IL-6, IL-8, Leptin, Adiponectin and BMI in group (A) and group (B) after treatment. TNF-α = tumor necrosis factor -alpha. IL-6 = Interleukin-6 IL-8= Interleukin-8 BMI= Body Mass Index T. Shimoda *1 , Y. Obase 2 , M. Imaoka 1 , R. Kishikawa 1 , T. Iwanaga 1 , 1. Fukuoka, Japan; 2. Nagasaki, Japan. Rationale: Serum high sensitivity C-reactive protein (hs-CRP) has been shown to be useful as a marker of airway inflammation in bronchial asthma. However, various factors have been reported to affect hs-CRP levels. We conducted a study in patients with mild bronchial asthma without complications to determine whether hs-CRP could serve as a useful indicator in assessing airway inflammation. Methods: The subject population consisted of 40 healthy volunteer (HV) controls, and 45 patients with bronchial asthma (BA). Induction of sputum with the use of inhaled 3% hypertonic saline and bronchial hyperresponsiveness test were performed, in addition to serum hs-CRP. Results: The log serum hs-CRP levels were higher in BA patients (2.49 ± 0.41) compared with healthy subjects (2.21 ± 0.39) (p=0.002). When receiver operator characteristic (ROC) analysis was performed to compare healthy subjects and BA patients, the sensitivity was 0.64 and specificity was 0.71 at a log serum hs-CPR of 2.3. The log serum hs-CRP levels were negatively correlated with FEV 1.0,%pred (r = -0.31, p = 0.04), positively with sputum eosinophils (r = 0.34, p = 0.02), but not with log PC 20 (r = -0.09, p = 0.56). Multiple regression analysis demonstrated that the log serum hs-CRP concentrations were significantly correlated with both eosinophils (p = 0.004) and neutrophils (p = 0.011) in sputum. Conclusions: Serum hs-CRP is useful in assessing airway inflammation in BA patients without complications such as heart disease, hypertension, hyperlipidemia, chronic obstructive pulmonary disease, and infections. Introduction: Studies have shown higher risk of cardiovascular disease (CVD) in asthma than non-asthma populations. However, there are limited data examining whether asthma severity is linked to CVD incidence within asthma populations. Methods: The Evaluating Clinical Effectiveness and Long-Term Safety in Patients with Moderate-to-Severe Asthma study (EXCELS) is a multicenter, prospective, 5-year, observational cohort study of patients with moderate-to-severe persistent asthma, including cohorts treated and not treated with omalizumab. This post hoc analysis examined the association between baseline asthma severity factors and longitudinal outcomes over 5 years in the nonomalizumab cohort. Cardiovascular-and cerebrovascular-related events were combined into a single endpoint of arterial thrombotic events (ATE), defined as the first of the following: cardiovascular death, myocardial infarction, ischemic stroke, transient ischemic attack, or unstable angina. An independent expert panel blindly adjudicated all ATEs. Asthma severity measures included spirometry, physician-assessed asthma severity, oral steroid usage, and the Severity of Asthma Score (SOA), a validated instrument that assesses asthma symptoms, asthma medication burden, and asthma emergency utilization history (range 0-28). Multivariable Cox models were used to assess risk of ATE for each individual asthma severity measure, controlling for potential confounders. Results: A total of n=2,829 patients in the non-omalizumab cohort were analyzed. Baseline mean age was 46.2±17.1 years, 66.5% were female, and 82.2% were white. Mean pre-bronchodilator % predicted forced expiratory volume (FEV 1 ) was 80.0±21.6 and mean SOA was 10.0±4.5. After controlling for potential confounders, lower pre-bronchodilator FEV 1 % predicted, higher SOA, and chronic oral corticosteroid usage were each statistically significantly associated with increased risk of ATE (Table) . Conclusions: Multiple asthma severity measures were associated with increased risk of ATE in this cohort. Further work is required to delineate mediators, but this analysis utilizing a large cohort followed over an extended timeframe provides some of the most robust evidence linking higher asthma severity to greater risk of cardiovascular and cerebrovascular outcomes. Adjusted Predictions for Time to First ATE Event (n=2,829 Patients) ATE, arterial thrombotic event; FEV1, forced expiratory volume in 1 second a) Analyses of FEV1 % predicted controlled for tobacco history (none, current, former) and education but not age, gender or race/ethnicity because these demographic factors are included in the calculation of percentage predicted. b) Controlling for age, gender, race/ethnicity, education, and tobacco history (none, current, former). Introduction: Little is known about the effect of omalizumab (OMA) continuation vs. discontinuation after its long-term use on patient-reported asthma symptom control. XPORT was the first rigorous study of long-term benefit of OMA continuation. Methods: XPORT was a randomized, double-blind, placebo-controlled withdrawal study of patients with moderate-to-severe persistent allergic asthma receiving long-term therapy with OMA for ≥5 years. Patients were randomized to either continue the same dose of OMA (n=88) or switch to placebo (PLB, n=88) and were followed every 4 weeks for 1 year. Patients completed the Asthma Control Questionnaire (ACQ) and the Asthma Control Test (ACT) at baseline (BSL) and every 4 weeks until week 52. High ACQ (range 0-6) and low ACT (range 0-25) scores indicate poor asthma symptom control. We compared changes in ACT and ACQ scores from BSL to week 52 and ACT and ACQ scores at each time point against the BSL (ANOVA tests). We compared the percentage of PLB and OMA patients (chi-square test) whose decline reached minimal important difference (MID) from BSL to Week 52 on ACT and ACQ. IRB approval and informed consent were obtained from all research subjects. Results: On average, patients had good asthma symptom control at the BSL (Table) . Patients randomized to continue OMA maintained better asthma symptom control than patients randomized to PLB. The mean change from BSL to Week 52 for ACT score was -2.88 for the PLB arm and -1.16 for the OMA continuation arm [p=0.0188]. The mean change from BSL to Week 52 for ACQ score was 0.63 in the PLB arm and 0.22 in the OMA continuation arm [p = 0.0039]. Compared to the BSL, the decline in asthma symptom control in ACT and ACQ was statistically significant for the PLB arm starting at week 4. There was no statistically significant change for the OMA arm at any time point (Table) . By week 52, the percentage of patients whose decline of asthma control reached MID comprised 28.4% (OMA) vs. 43.2% (PLB) on ACQ (p = 0.041) and 23.9% (OMA) vs. 39.8% (PLB) on ACT (p=0.024). Conclusions: Patients with allergic asthma who continue on OMA therapy maintain asthma symptom control. However, patients who discontinue OMA therapy report significant decline in asthma symptom control, and this is evident as early as week 4. ACQ and ACT scores by study visits and treatment arms PLB = placebo; OMA = omalizumab. High ACQ and low ACT scores indicate poor asthma symptom control. * P < 0.05. ** P < 0.01. P-values were derived from ANOVA, comparing against baseline. Background: Multiple studies have shown inadequate parental knowledge about asthma is common among pediatric patients seen in Emergency Rooms (ER) for acute asthma. Furthermore educational interventions have resulted in improvements in both the asthma knowledge of parents, as well as disease control of patients. However in adult populations there are conflicting data regarding the relationship between asthma knowledge and disease control. Methods: We measured asthma knowledge with the Asthma Self-Management Questionnaire, a validated 24 item true/false instrument, in 49 adult asthmatics while visiting any of 3 primary care clinics at a single tertiary referral center. Asthma control was measured by Asthma Control Test (ACT), mini-Asthma Quality of Life Questionnaire (m-AQLQ), as well as patient reported: monthly short acting beta-agonist (SABA) use, ER visits for asthma in the past year, and lifetime hospitalizations for asthma. Other demographic, socioeconomic, including literacy as measured by the Newest Vital Sign (NVS), and asthma care data were also collected. Results: Asthma knowledge was correlated with asthma control as measured by monthly SABA use (r=-0.29 p=0.05) but was not with ACT (r=0.17, p=0.24), m-AQLQ (r=0.19, p=0.19), ER visits (r=-0.13, p=0.13), and hospitalization (r=-0.11, p=0.47). Asthma knowledge was correlated with specialist referral (r=0.41, p=0.003) and patient literacy (r=0.37, p=0.01), but not with the patient's reported highest level of formal education (r=0.19, p=0.20), patient income (r=0.08, p=0.60), time since asthma diagnosis (r=0.22, p=0.13), and controller medication prescription (r=0.04, p=0.8). Discussion: In this population asthma knowledge was correlated with SABA use, patient literacy and specialist referral, but did not correlate with other measures of asthma control, socioeconomic status, and asthma management. However this was a small survey conducted in a single facility and the study population had significantly higher scores than the population in which the knowledge measurement tool was validated. Consequently further investigation into adult patient knowledge and asthma control is warranted. Background: Recently published European Respiratory Society (ERS) and American Thoracic Society (ATS) asthma guidelines provide a new definition of severe asthma based on control measures and medication use. The relationship between asthma severity and blood eosinophils (EOS) was examined using this new guideline definition. Objective: To study the association of asthma severity defined by ERS/ATS guidelines with blood EOS levels. Methods: Records of patients at least 12 years of age with at least two medical claims with a primary or secondary diagnosis of asthma defined by ICD-9-CM code 493.xx between January 2004 and July 2011, were extracted from the EMR-Claims+ database (eMAX Health, White Plains NY) containing electronic medical records linked to insurance claims across 6 Midwestern states in the U.S. The date of first diagnosis was defined as the 'index date'. Patients were required to have at least 1 peripheral blood EOS test in the 12 month 'assessment' period following the index date. Patients' asthma was classified as severe or non-severe using intensity of treatment and level of asthma control during the assessment period, as recommended by the 2014 ERS/ATS guidelines. An elevated EOS was defined as > 400 cells/mL. Chi-square tests were utilized to compare the proportion of patients with elevated EOS between the two severity groups. Multivariable logistic regression was used to assess the relationship between asthma severity and EOS, after controlling for potential confounders. Results: Among the 2,164 patients with asthma and EOS test results in the assessment period, 9% (n=184) had severe asthma according to ERS/ATS guidelines. A significantly greater proportion of patients with severe asthma had elevated EOS, compared to patients with non-severe asthma (30% vs. 19%, p=0.0002). In multivariable analysis, severe asthma was associated with an 83% increased odds of elevated EOS (Odds Ratio = 1.83, p=0.0005), compared to those with non-severe asthma after adjusting for differences in patient demographic characteristics and comorbidities. Conclusion: An elevated EOS is associated with nearly double the odds of severe asthma. Background: Prior to 2011, patients admitted with asthma to this tertiary care children's hospital received bedside education from a registered respiratory therapist (RRT) with variable intensity and consistency. To address this problem, an Inpatient Asthma Class (IAC) was implemented three days a week in 2011, which was increased to five days a week in 2014. Our specific aim was to evaluate the relationship between expanding parental access to an IAC and RRT time spent on bedside education. Methods: ORI determination was obtained for non human subjects research. The study population included children with asthma admitted from February 2011 to May 2014. RRT's were asked to document in the electronic medical record (EMR) the amount of time spent on bedside education with each family as: 〈15 minutes, 15 minutes, 30 minutes, or 〉30 minutes. Data was extracted from the EMR to investigate RRT time spent on bedside education when the IAC was offered three days a week, compared to five days a week. Analyses were performed in Stata 11.2 and Excel (.xlsx) using chi-squares and differences in proportions. Results: About 100 RRT's provided bedside education for 2546 patients and their families during the study period. When the IAC was offered three days a week, 21% (469/2197) of patients did not have documentation of time spent on education, similar to 26% (91/349) who did not have documentation when the IAC was offered five days a week. When the IAC was offered three days a week, 46% (787/1728) of parents attended; whereas 61% (157/258) attended when the IAC was offered five days a week (p〈0.0001). When the IAC was offered three days a week, 33% (261/787) of families required 〈15 minutes of bedside education, compared to 48% (75/157) when the IAC was offered five days a week (p=0.0005). Overall, 8% (75/944) of families required ≥30 minutes of bedside education with parental attendance in the IAC, compared to 17% (172/1042) of patients without parental attendance in the IAC (p〈0.0001). Conclusion: Expanding the IAC from three days a week to five days a week increased parental attendance, resulting in a reduction in bedside education time. This time-savings has allowed reallocation of RRT's to more urgent clinical needs throughout the hospital. The increasing importance of cost-efficient, value-based care supports the future provision of educational programs such as the IAC. Background: The effect of budesonide/formoterol (BUD/FM), BUD, or FM on fixed airflow obstruction (FAO) and withdrawal due to predefined asthma events (PAEs) is unclear. Methods: This was a post-hoc analysis of a 12-week, randomized, placebo (PBO)-controlled study (NCT00652002) of patients aged ≥12 years with moderate-severe asthma. After 2-week run-in with twice-daily BUD 160 mg via pressurized metered dose inhaler (pMDI), patients received twice-daily BUD/FM pMDI 320/9 mg, BUD pMDI 320 mg, FM 9 mg via drypowder inhaler, or PBO. Worsening asthma event criteria were predefined (PAEs; Drugs. 2006;66:2235-54). Postbronchodilator FAO status was assessed at screening, weeks 2, 6, and 12 via forced expiratory volume (FEV 1 )/forced vital capacity (FVC) ratio for patients who completed the study. This analysis excluded patient withdrawals for any reason before week 2. Persistent FAO− (FEV 1 /FVC ≥ lower limit of normal [LLN] ) and persistent FAO+ (FEV 1 /FVC 〈 LLN) patients retained their FAO status at all visits. FAO status of variable patients was not consistent on all visits. FAO status and withdrawals due to PAEs were assessed. Results: In 258/389 patients (66%), FAO status was persistent throughout the study (135 FAO− and 123 FAO+). The remaining 131 patients (34%) had variable FAO, changing status after screening. The percentage of patients with persistent FAO− (44%, 37%, 28%, and 30%), persistent FAO+ (30%, 29%, 35%, 33%), and variable FAO (26%, 34%, 38%, and 37%) differed in the BUD/FM, BUD, FM, and PBO groups, respectively. Overall, persistent FAO− patients had fewer PAEs than FAO+ or FAO variable patients. The numbers of patients who withdrew due to PAEs in the persistent FAO− (n = 2, 4, 3, and 7), persistent FAO+ (n = 1, 4, 15, and 15), or FAO variable (n = 6, 8, 15, and 12) classifications also differed for the BUD/FM, BUD, FM, and PBO groups, respectively. Conclusions: Patients with moderate to severe asthma treated with BUD/FM are most likely to remain persistent FAO−, least likely to become FAO variable, and least likely to withdraw from the study if FAO+. Patients who were persistent FAO− vs persistent FAO+ or FAO variable had fewer withdrawals due to PAEs. Irrespective of FAO status, patients receiving BUD/FM experienced the fewest withdrawals due to PAEs. Supported by AstraZeneca LP Background: The effects of fixed airflow obstruction (FAO) on peak expiratory flow (PEF) and rescue medication (RM) use with budesonide/formoterol (BUD/FM) in patients with moderate to severe asthma is unclear. Methods: In a 12-week, double-blind, double-dummy, placebo (PBO)-controlled study (NCT00652002), moderate to severe asthma patients aged ≥12 years were randomized to twice-daily BUD/FM via pressurized metered dose inhaler (pMDI) 320/9 mg, BUD pMDI 320 mg, FM dry powder inhaler 9 mg, or PBO after a 2-week run-in with twice-daily BUD pMDI 160 mg. In a post-hoc analysis, postbronchodilator FAO status was assessed via forced expiratory volume in 1 second/forced vital capacity (FEV 1 /FVC) ratio at screening and weeks 2, 6, and 12 for patients who completed the study, excluding patient withdrawals for any reason before week 2. Persistent FAO− (FEV 1 /FVC ≥ lower limit of Introduction and Aim: Asthma is chronic respiratory disease characterized with recurrent episodes where environmental factors are important. An asthmatic patient whose clinical and radiological findings persisted after lovebird contact was presented. Case: A nine-years-old allergic asthmatic patient was admitted due to persistant cough and wheezing for 1 months. Her complaints increased after living with a lovebird at home. Her father, aunt, and cousins had asthma, tuberculosis (TB) contact wasn't mentioned. Bilateral roncus and prolonged expiration were found in examination. Inflammation in chest X-ray and mite-allergy in prick test (eosinophil 3.9%, ImmunglobulinE 92 mg/dl) were determined. Asthma and allergic rhinitis were treated with inhaled steroid, longacting beta-2 agonist, leucotrien antagonist, nasal steroid. Macrolid was given for atypical Mycobacteria. Symptoms persisted after two weeks of these therapies. Computarized tomography revealed atelectasis, infiltrations and frostedglass appearance. Extrinsic allergic alveolitis due to lovebird was thought. Bird feather specific IgE, Aspergillus antigen, Chlamydia and Mycoplasma serology, and sputum cultures were negative, Quantiferon test was positive. Fiberoptic bronchoscopy was normal, ARB in BAL and gastric aspirates were negative. Anti-TB therapy was begun due to resistant clinical and radiological symptoms and positive Quantiferon test. During the follow-up, Mycobacterium complex was obtained from BAL culture. Family screening was negative, after a while her adolescent neighbour had TB was told by the family. Clinical and radiological complete improvement was seen after therapy. Conclusion: The diagnosis of TB disease should be excluded in the patients where clinical and radiological symptoms and findings are resistant to therapy. Poorly controlled asthma is diagnosed when despite high doses of inhaled corticosteroids daily plus short and long acting β-2-agonists, and other therapies, symptoms interfere significantly with daily activities, frequent exacerbations need to be solved with the administration of corticosteroids, frequent emergency room visits or hospital admissions and the need for oral corticosteroids. Material and Methods: We performed a pilot, prospective, open study from June to December 2013. We included 30 asthmatic patients who signed informed consent and were over 18 years old. They were clasified according to GINA in partly controlled or uncontrolled and treated with inhaled steroids at high doses plus long acting β-2-agonist. In the first visit we registered a spirometry with bronchodilator, asthma control test of 5 questions (ACT) and the quality of life of asthma (mini-AQLQ). We then indicated ICS and LABA plus tiotropium 2.5 mcgs a day or roflumilast 500 mcgs a day or theophylline 200 mg every 12 hours. ACT were evaluated and mini-AQLQ once a month via phone and on 2 visits at 3 and 6 months, spirometry with bronchodilator at 6 months and peak flow meter measured at home. The main objective was to measure the ACT, the second objective was to assess mini-AQLQ, FEV1 and peak flow. Results: Mean age was 48 years. 65% were women. ACT <16: 10 uncontrolled patients and between 16 and 19 ACT: 20 patients partially controlled. The mean duration of asthma was 11 years. Fifteen patientys received tiotropium, 10 patients Roflumilast and 5 theophylline. 3 patients discontinued treatment during the 6 months. In all 3 groups improved ACT and mini-AQLQ (p <0.0001). The total values of lung function with tiotropium improved FEV1 2.46 ± 0.89 L at baseline to 2.60 ± 0.92 L at 6 months (p <0.0001); with roflumilast 2.50 ± 0.90 L at baseline to 2.63 ± 0.96 L at 6 months (p <0.0001); and 2.43 ± 0.84 theophylline L at baseline to 2.53 ± 0.91 L at 6 months (p <0.0001). PEF with tiotropium increased from 5.76 ± 2.38 to 6.22 ± 2.47 L / s (p <0.0001); with roflumilast increased from 4.65 ± 2.22 to 5.11 ± 2.32 L / s (p <0.0001); and theophylline increased from 5.13 ± 2.17 to 6.01 ± 2.31 L / s (p <0.0001). Conclusion: It is premature to ensure that these drugs are safe and effective. We conclude that adding tiotropium, theophylline and roflumilast achieves a better disease control, increases FEV1, PEF and improves quality of life. This a case report of a 59 year old female presenting with a persistent severe cough of 10 months duration. She had multiple evaluations for asthma, GERD, and treament with multiple inhalers and antibiotics without response. History was negative for occupational exposures to known agents causing hypersensitivity pneumonitis such as gases, paints (TDI) or other agents. There was no history of emphysema in non smokers in her family and she is a lifelong non smoker. When asked about pets previously she stated that she had no cats or dogs. When questioned in detail about bird exposure such as pigeons close to her windows, etc,she stated that she had a Cockatiel that she purchased 12 months before her presentation to our office. On further history she stated that she was the one who cleaned the cage of droppings and that no mask was worn. On physical exam she had no dyspnea she did have cough ( with no inspiratory stridor), no clubbing, pO2=95, she had diffuse crackling breath sounds on auscultation. Her Spirometry showed FVC=59% predicted and FEV1=69% predicted. We ordered chest xray, arranged for a Spirometry with DLCO gas exchange, titers for Chlamydia pneumonia and Mycoplasma pneumonia,hypersensitivity pnuemonitis panel plus titers and precipitins for parrot/cockatiell ( if available) and alpha 1-antitrypsin was considered if the above was negative. Unfortunately due to the expense after insurance the patient declined the testing. She removed the Cockatiel from the home and within 3 months her cough totally abated. She declined followup spirometry and was lost to followup. In patients with chronic respiratory issues or chronic cough a thorough investigation of pets, including birds, and fish ( we had one patient with HSPneumonitis to silkworm powder used to feed fish). In this case she refused to do diagnostic studies but our clinic suspicion was that her Cockateil and her exposure to the cage droppings were considered highly likely to be the cause. There are studies showing Cockatiel to be an antigen stimulating precipitins. McClusky, et.al. published on a case where a similar patient showed positive precipitating antibody to cockatiel dropping mix (CDM) . A.B. Vutikullird 1 , M. Gillespie 2 , S. Song 2 , J. Steinfeld *2 , 1. Cypress, CA; 2. Frazer, PA. Introduction: Fluticasone (Fp), an inhaled corticosteroid, was approved in 1993 for the maintenance treatment of asthma. It has well-established safety and efficacy profiles and is available as a dry powder inhaler formulation and a hydrofluoroalkane inhalation aerosol. In the present study, Fp is delivered via a novel, inhalation-driven multidose dry powder inhaler (MDPI) that eliminates the need to coordinate actuation with inhalation. Methods: This was a singlecenter, open-label, randomized, 3-period crossover, single-dose study in healthy Japanese and Caucasian subjects aged 20-45 years, inclusive. Subjects (N=30) were randomized to 1 of 6 treatment sequences including combinations of 4 inhalations of Fp MDPI 100 mg (total dose of 400 mg), Fp MDPI 200 mg (total dose of 800 mg), and Fp-DISKUS ® (GlaxoSmithKline, Research Triangle Park, NC) 100 mg (total dose of 400 mg). The primary objective was to assess pharmacokinetics (PK) (maximum plasma concentration and time, terminal rate constant and half-life for plasma elimination, area under concentration-vs-time curve) for each treatment; safety and tolerability also were assessed. Results: Over the range of all Fp doses evaluated, PK parameters were similar in Japanese and Caucasian subjects. Differences were observed between the systemic exposures produced by each of the 3 doses, with the Fp plasma concentrationvs-time curves being highest for the Fp MDPI 800 mg total dose and lowest for the Fp DISKUS 400 mg total dose ( Figure) . The most frequently occurring adverse event was presyncope, which occurred in 2 Caucasian subjects, 1 treated with Fp MDPI 400 mg total dose and 1 treated with Fp MDPI 800 mg total dose. There were no clinical laboratory, electrocardiographic, vital signs, or physical examination findings that were considered clinically significant. Conclusions: The PK profiles for Fp MDPI 400 mg, Fp MDPI 800 mg, and Fp DISKUS 400 mg were similar in Japanese and Caucasian subjects; however, there were differences in the systemic exposures dependent on total dose, with Fp MDPI 800 mg being highest and Fp DISKUS 400 mg being lowest. The safety and tolerability profiles of all treatments were consistent with the known profile of Fp. This study was sponsored by Teva Pharmaceuticals Introduction: A novel, inhalation-driven, multidose dry powder inhaler (MDPI) has been developed that eliminates the need to coordinate actuation with inhalation and may reduce administration errors in comparison with conventional metered-dose inhalers. Studies show that albuterol (AB) is effective and well tolerated for the prevention of exercise-induced bronchoconstriction (EIB). Methods: This single-dose, double-blind, 2-way crossover study (NCT01791972) randomized adolescents and adults with EIB (≥20% fall from pre-exercise challenge FEV 1 ) to treatment sequences AB MDPI 180 mg/placebo (PBO) MDPI (n=19) or reverse sequence (n=19). IEC/IRB approval and informed consent from all subjects were obtained. FEV 1 was measured at 30 minutes post-dose, 5 minutes before treadmill exercise challenge (baseline) and 5, 10, 15, 30, and 60 minutes after exercise challenge. The primary efficacy endpoint was maximum percentage fall from baseline in FEV 1 up to 60 minutes post-exercise challenge. Secondary efficacy endpoints included percentage of patients whose maximum percentage fall from baseline FEV 1 post-exercise challenge was <10% (protected by treatment) and time to recovery (from exercise challenge completion to the first measured post-challenge FEV 1 within 10% of the pre-challenge baseline FEV 1 ). Results: Age, gender, and baseline FEV 1 were comparable between subjects in both sequences. Mean maximum percentage fall in FEV 1 within 60 minutes post-exercise challenge for AB MDPI was 6.21±1.44% (95% CI, 3.28%-9.14%) vs PBO, 22.38±1.44% (95% CI, 19.44%-25.31%; p<0.0001). Thirty-two (84.2%) AB MDPI subjects had a <10% maximum FEV 1 fall post-exercise challenge vs 6 (15.8%) PBO subjects. Protection with AB MDPI was significant (p<0.001) vs PBO within 5 minutes and maintained through 30 minutes; recovery was complete for both groups at 60 minutes. Median time to recovery for AB MDPI was 5.07 minutes vs PBO, 30.07 minutes (p<0.0001). Adverse events (AEs) included severe migraine (n=1, PBO) and moderate sinus bradycardia (n=1, AB MDPI). No serious AEs, discontinuations due to AEs, or deaths occurred. Conclusions: AB MDPI provided clinically significant protection from EIB in adolescents and adults with a history of EIB. There were no safety issues with short-term AB MDPI use. This study was sponsored by Teva Pharmaceuticals. Introduction: While recognized as the most effective maintenance treatment for mild-to-moderate persistent asthma, inhaled steroids have the potential to cause growth suppression in children. Several studies performed with inhaled corticosteroids demonstrated an impact on growth velocity (ranging from -0.3 cm/yr to -1.8 cm/yr) with recent studies suggesting that this effect is not reversible once treatment with the inhaled steroid is stopped. Two 1-year trials were performed with flunisolide HFA (Aerospan), a small-particle inhaled corticosteroid with a built-in spacer for the treatment of asthma in asthmatics 6 years and older, to evaluate growth velocity changes in pre-pubescent children 4 to 11 years of age. Methods: The first study was an open-label, randomized, 1-year safety study of flunisolide HFA, inhaled beclomethasone (BDP) CFC, and inhaled cromolyn (as a negative control) in children 4 to 11 years of age with mild-to-moderate asthma. The second study was a double-blind randomized 1-year safety study of flunisolide HFA and placebo in children 4 to 9 years of age with mild-to-moderate asthma. Each study was analyzed similarly for growth velocity (via stadiometry assessments) and change in height over 1 year (measured as cm/yr). The dose of flunisolide HFA in both studies was 160 mcg (2 puffs) twice daily, which represents the maximum FDAapproved dose of flunisolide HFA for children. Results: In the open-label study, 206 patients age 6-11 years, were analyzed for growth and growth velocity. Mean changes from baseline height were 6.2 cm for the flunisolide HFA and cromolyn groups versus 5.1 cm for the BDP group. Mean growth velocity was 6.2 cm/yr for the flunisolide HFA group versus 5.3 cm/yr (BDP) and 6.9 cm/yr (cromolyn). In the double-blind study, of 218 evaluable patients age 4-10 years, the mean growth velocity was 6.01 cm/yr for flunisolide HFA versus 6.19 cm/yr for placebo, a non-significant difference (P=.425). Conclusion: The results from two trials demonstrated that flunisolide HFA did not result in significant growth suppression in pre-pubescent pediatric asthma patients 4 to 11 years of age when compared to placebo or the negative control (cromolyn). Improving asthma control has focused on potentially modifiable clinical and behavioural characteristics including correct inhaler technique, allergic rhinitis (AR), adherence and smoking. This research aims to establish these relationships with asthma control, outcomes and quality of life. A US crosssectional survey of adult asthma patients seeking routine care, informed Partial Least Squares Path Modelling to quantify inner model relationships between latent variables of patient-reported satisfaction of drug delivery, device functionality, device feedback, concomitant AR, adherence (Morisky Medication Adherence Scale), smoking history, patient-reported outcomes (Asthma Control Test, Jenkins Sleep Questionnaire, EuroQol-5D-3L) and physician-reported asthma exacerbations in last 12 months. Patients not receiving inhaled maintenance therapy were excluded. 243 patients were included. All actual variables were positively correlated with the appropriate latent variable. Cronbach's Alpha, a measure of consistency for a group of variables, measured at least 0.659 for latent variables indicating that the hypothetical variables of inhaler satisfaction, smoking history, concomitant AR, adherence and outcomes were well reflected by the composition of the actual variables in the dataset. Cross-load-ings were also supportive of the hypothesized outer model. Better patient outcomes were significantly associated with patient satisfaction regarding drug delivery attributes related to the inhaler (p=0.002). A negative smoking history coupled with absence of concomitant AR were also significantly associated with better patient outcomes (both p<0.001). Improved adherence did result in enhanced outcomes, but this relationship was not statistically significant (p=0.060). The R 2 value for outcomes was 14.6% and pseudo goodness of fit, measuring overall prediction performance of the path model, was 20.56% indicating a fifth of the variance was explained by this model. Improving patient satisfaction with inhaler drug delivery parameters represents one modifiable aspect of asthma management contributing to better control. Interventions based on improved recognition of impact of severe AR, allergy phase and smoking on patient's asthma control are also likely to have a positive impact on asthma patient health-related outcomes. A.M. Koatz * , Buenos Aires, Argentina. Introduction: Recurrent acute infections of the upper and lower airways are frequent and often require multiple therapies. This is especially true for pathologies such as allergic rhinitis and/or asthma and/or COPD. The etiology of recurrent infections is generally viral, but the additional complication of bacterial infection worsens the symptoms of the underlying pathology. For these reasons, preventive measures should be considered when treating acute infections. Methods: a randomized, double-blind, placebo-controlled clinical trial was conducted. Patients aged 16-65 years who had not received a lysate vaccine in the previous year were included. Eighty-four (84) patients were randomly assigned to 2 groups of 42 subjects each one :-Group A (active) -receive either OM-85 bacterial lysate vaccine capsule or equivalent placebo. Capsules were administered daily for 10 consecutive days per month for a duration of 3 consecutive months..Group C (healthy controls) serologic studies were performed in 10 subjects. Serum and secretory IgA levels were determined before and after treatment along with symptom score and the number of infections and exacerbations. Written informed consent was obtained from all study participants. Results: Eighty-four (84) patients were enrolled and 42 patients that received OM-85 had a decrease in the number of recurrent infections from 82% to 45.5% (p< 0.05) compared to 78.9% in the placebo group. Similarly, the exacerbations of the underlying pathology decreased from 65.7% to 34.9% with OM-85 versus 62.3% in the placebo group (p<0.05) There was also a substantial reduction of the symptoms of the underlying pathology. (p<0.01) A significant increase in serum and secretory IgA levels was observed only in the OM-85 group (both p<0.001) during which parallels the 3 cycles of therapy. Conclusion: This study demonstrated a clinical benefit of OM-85 for reducing reinfections and exacerbations in patients with allergic rhinitis and/or asthma and/or COPD. The use of OM-85 lead to an increase in serum and secretory IgA levels which provides a new perspectives regarding the immunological mechanism of action, namely immunostimulation and immunodesviation. Further investigation is warranted to better understand these encouraging outcomes. Introduction: It is important to identify clinical features associated with asthma for the development of new interventions to be most effective. Our clinical observation suggested the hypothesis that the proportion of overweight/obesity is significantly higher in difficult to control (DTC) than in well-controlled asthmatics. Methods: This was a retrospective chart review of 100 patients, aged 5 to 18 years, who were seen in a CHKD or one of the CSG Allergy outpatient clinics between 5/1/2013 and 3/31/2014, and diagnosed with asthma. Cases (n = 50) were identified as severe persistent DTC asthmatics based on their required high dose of ≥1000 mg/day of inhaled corticosteroids (ICS). The control group (n = 50) included subjects with well-controlled mild or moderate persistent asthma defined by a dose of ≤ 500 mg/day of ICS. A subject's weight status was classified by the BMI percentile for age as normal weight (BMI < 85%), overweight (BMI 85-94%), or obese (BMI ≥ 95%). The chisquare test was used to assess the relationship between overweight / obesity status and DTC asthma status. IRB and HRC approval and waivers of consent and authorization to access protected health information were obtained. Results: The BMI percentile in the DTC asthma group was significantly higher than in the well-controlled group (75.74 ± 25.63 vs. 51.69 ± 24.43, p < 0.001). 36 % of the DTC asthmatics were obese (vs. 0% of the well-controlled asthmatics, p < 0.001), 14% overweight (vs. 10% of the well-controlled asthmatics, p = 0.99) and 50% normal weight (vs. 90% of the well-controlled asthmatics, p < 0.001). Mean age in the DTC asthmatics was 13.2 ± 3.3 vs. 10.0 ± 3.2 in the well-controlled group (p < 0.001). Notably, a significant difference was observed in the race between the DTC and the well-controlled asthma group. 74% of the DTC asthmatics were African American (AA) (vs. 30% AA in the well-controlled asthma group, p < 0.001), 14% were White (vs. 66% in the well-controlled group, p < 0.001). There was no significant difference in gender between the DTC and well-controlled asthmatics. (Table 1) . Conclusion: The results of this study suggest that there is a significant association between obesity and severe persistent DTC asthma. This finding underlines the need for new treatment approaches for DTC asthmatics involving a simultaneous treatment of asthma and obesity. Introduction: High dose inhaled corticosteroids have been increasingly recognized to cause adrenal suppression. Patients reported in the literature typically have presented in the first decade of life. We report on the first case of mometasone furoate (MF) induced adrenal suppression on MF therapy. Case Description: A sixteen-year-old female with asthma and allergic rhinitis since the age of five was initially maintained on a regimen of FP 500mcg per day and MF nasal spray 50-100 mcg per day. As she had uncontrolled symptoms she was changed to MF/formoterol fumarate 800/ 20 mcg per day in addition to her nasal spray. She was more stable in follow-up but presented with increasing fatigue, weight loss and nausea. She also reported striae. Extensive initial hospital investigations were normal. A random glucose was 5.0 (3.4-7.0) and an AM cortisol was reported at < 50 nmol/L (170-540). She was started on hydrocortisone therapy and changed to ciclesonide, as well as montelukast and formoterol. Her energy and weight have improved after initiation of replacement hydrocortisone therapy with plans for an ACTH stimulation test after discontinuation of oral corticosteroids. Discussion: While inhaled FP has been most commonly implicated in adrenal suppression, to our knowledge this is the first reported case of symptomatic MF-induced adrenal suppression. Despite having high protein binding, we hypothesize that the pharmacokinetics of MF with a high receptor affinity, long half-life and decreased hepatic clearance when used at a high dose result in similar adrenal suppression as has been reported in FP. Physicians need to remain aware of the possibility of adrenal suppression in any patient on high dose inhaled corticosteroid. L. Greos *1 , J. Corren 2 , N. Ruiz 3 , J. Karafilidis 3 , 1. Centennial, CO; 2. Los Angeles, CA; 3. Somerset, NJ. Introduction: Inhaled steroids (ICSs) are the most effective maintenance treatment for mild-to-moderate persistent asthma. Flunisolide HFA, a small particle ICS with a built-in spacer, is approved for treatment of asthma in patients 12 years and older at doses of 160 mcg (2 puffs) and 320 mcg (4 puffs) BID. Efficacy and safety comparisons to each patient's prior ICS offer important perspective. Methods: This was a multi-center, double-blind, placebo-controlled trial consisting of a 2-week run-in with flunisolide CFC (500 mcg BID), followed by double-blind treatment with placebo, flunisolide HFA (80, 160 and 320 mcg BID), or flunisolide CFC (250, 500 and 1000 mcg BID) for 12 weeks in patients 12 and older with mild-to-moderate asthma. The primary efficacy endpoint was change from baseline in % predicted FEV1. A retrospective analysis of the primary efficacy endpoint by baseline ICS (all patients were previously treated with an ICS) was performed to evaluate potential differences in efficacy in patients previously treated with another ICS. The most commonly used previous ICSs were: fluticasone (FP-195 patients), beclomethasone (BDP-190 patients), triamcinolone acetonide (TAA-174 patients) and flunisolide (FLN-98 patients). Patients were required to be on a stable dose of ICS for at least 30 days prior to trial entry. Results: Of the 669 randomized patients, 548 (82%) completed the trial. Compliance was >90% at all visits in each treatment group. Patients experienced about a 12% improvement in FEV1 after the 2week run-in with flunisolide CFC. After 12 weeks of treatment, patients treated with flunisolide HFA 160 mcg BID maintained the 12% improvement achieved during run-in, patients treated with 320 mcg BID had a 0.4% improvement over run-in, and patients treated with placebo had a 4.5% decrease (p<0.007; active HFA treatments vs placebo). LS mean changes from baseline in % predicted FEV1 did not significantly differ based on previous ICS, with the most significant improvements seen with the 160 and 320 mg BID doses of flunisolide HFA compared with placebo. Conclusion: The results from this retrospective evaluation by baseline ICS suggest that the efficacy with flunisolide HFA 160 and 320 mcg BID is at least equivalent to other ICSs, as demonstrated by similar % improvements in FEV1 for each of the baseline ICS subgroups. Rationale: Foreign body aspiration (FBA) occurs more commonly in children than adults. Diagnosing FBA in adults with allergic asthma and allergies is challenging because patients may not remember choking and there may be confounding symptoms. Case: A 41 year old female with history of allergic asthma, rhinitis, and nasal polyps was seen for evaluation in March 2014. She complained of cough and intermittent wheezing that had worsened since Thanksgiving 2013. Her symptoms began with bronchitis that developed into chronic wheeze and productive cough; she had minimal improvement after treatment with antibiotics and oral corticosteroids. Chest x-rays were clear in January and March, 2014. She was started on inhaled fluticasone-salmeterol by a pulmonologist and referred for allergy evaluation after minimal improvement of respiratory symptoms. She had a course of allergen immunotherapy in the early '90s and surgery to remove nasal polyps in 1992. Findings: On initial visit, she had end-expiratory wheezing auscultated in the right lung. FEV1 was 100%, and FeNO was 30 ppb. Allergy skin tests were positive to multiple inhalant allergens. In-office nasopharyngoscopy showed purulent sinus discharge and scarring from previous nasal surgery. She was treated with cefdinir and started on inhaled budesonide, montelukast, mometasone intranasal sprays, sinus irrigations, and allergen immunotherapy. Her cough improved somewhat after cefdinir, but she reported a persistent nighttime cough and had end-expiratory wheeze on exam on follow-up. In May 2014, CT chest showed calcified granuloma in the intra-inferior aspect of the right lower lobe. Subsequent bronchoscopy identified a foreign body (suspicious for peanut) that was removed from the right bronchus intermedius. Pathology reported a foreign degraded organic material. The patient subsequently recalled an episode of choking on peanuts in January 2014. Conclusion: This is a report of an adult with previously diagnosed allergic asthma, rhinitis, and nasal abnormalities that confounded the diagnosis of FBA. Although initial history and diagnostic evidence did not suggest FBA, FBA should be considered in adult patients with persistent wheezing unresponsive to asthma therapy. CT imaging and bronchoscopy are sometimes necessary for a conclusive diagnosis. M. Chen *1 , E. Rael 2 , 1. State College, PA; 2. Hershey, PA. Rationale: Eye symptoms are commonly seen in patients with allergic asthma and the combination can have significant effects on quality of life and work productivity. Common allergic eye conditions include allergic conjunctivitis, vernal keratoconjunctivitis and atopic keratoconjunctivitis. Here we report amongst a cohort of asthmatics at a university based allergy, asthma, and immunology specialty clinic, correlations between subjective eye symptoms and asthma symptoms. Methods: After informed consent and IRB approval, 370 asthmatics, recruited to the study between 2011 and 2014, underwent clinical assessment including review of the Eye Symptom Score as part of the mini RQLQ score, the total mini RQLQ score, and ACQ quality of life asthma symptom score to determine whether there is a correlation between eye symptoms and asthma severity. The mean assessment divided the cohort by ACQ scores into group 1, low risk asthmatics for future asthma events in the next 2 weeks as demonstrated by ACQ < 1.50 and group 2, high risk asthmatics for future asthma events in the next 2 weeks as demonstrated by ACQ > 1.50. Results: Group 1 asthmatics represented 243 subjects and group 2 asthmatics represented 127 subjects. Subjects in group 1 had a mean mini RQLQ eye symptom score of 4.4 in comparison to a higher score of 7.1 amongst group 2 subjects. The average total mini RQLQ score in group 1 subjects was 25.9, versus 41.9 amongst group 2 subjects. Conclusions: This study shows that patients who have higher immediate risk for asthma flares concurrently have higher ocular and total upper airway symptoms scores further validating the one airway hypothesis. Further studies are necessary and future research could assess whether there are different ocular phenotypes associated with asthma severity. E. Egea *1 , G. Garavito de Egea 1 , L. Fang 1 , G. Egea 1 , L. Visbal 1 , N. Lecompte 1 , J. Escamilla 2 , 1. Barranquilla, Colombia; 2. Cartagena, Colombia. Background: Humans have the ability to synthesize vitamin D during the action of ultraviolet (UV) radiation upon the skin. The term "vitamin D insufficiency" has been associated with asthma. However, little is known about the link between total and serum specific IgE levels and vitamin D in asthmatic children. Today dust mite allergens and cockroach are the most important sources of clinically relevant allergens sensitizing patients with asthma in the tropics, Objective: To investigate the relevance of Vit D levels on the total Ig E and allergen-specific sensitization(Cr and Mite) in asthmatic children living a poorer communities within a Barranquilla, a city on the Colombian Caribbean Coast. Method: A case-control study with 1000 individuals, which included 500 asthmatic children and 500 healthy children from the Colombian Caribbean coast was made. Serum 25-hydroxy vitamin D was measured by a EIA. Total IgE, specific IgE to Dermatophagoides farinae-D f-and Blomia tropicalis-B t.and Periplaneta americana -Cr-were determinated by ELISA (ImmunoCAP ® Phadia AB, Sweden technology). The independent effects of these variables were estimated from obtaining odd ratios (OR) trough logistic regression models adjusted for sex and age. Results: Elevated total IgE (> 100UI/mL) were observed in both study group. Showing as a risk factor for susceptibility to childhood asthma (OR = 1.63, 95% CI = 1.08 -2.46). Serum levels of vitamin D were found normal or insufficient in both groups (52.21 ng / mL and 52.2 ng / mL, P = 0.907). Also, insufficient vitamin D was associated with elevated levels of Total Ig E (15% vs 8.7%, P = 0.037); showing a negative correlation (Spearman r = -0.28, p = 0.00). However, our data is not clear regarding what effect it may have the insufficient levels of vitamin D on the IgE immune response (OR = 1.84, 95% CI = 0.9 -3.6). On the other hand, elevated Total IgE levels were associated with specific IgE sensitization to B. tropicalis, D. farinae and P. americana. Conclusion: Despite the growing evidence of the role of vitamin D in incident asthma and asthma exacerbation, studies are not all consistent Taking together our results are showing that insufficient serum levels of vitamin D is not a risk factor for developing asthma in poores comunities children of the Caribbean area. Introduction: A previous study concluded that blood eosinophil counts in patients with asthma could be used as an indicator of prospective asthma outcomes. We aimed to identify risk factors associated with elevated blood eosinophil counts in patients with asthma from UK primary care clinical records. Methods: Retrospective, observational study using the Clinical Practice Research Datalink and the Optimum Patient Care Research Database. Patients were aged 12-80 years with asthma and no other chronic respiratory disease. Study period was 1 year before (baseline period) date of last recorded blood eosinophil count. Comparison at date of last recorded blood eosinophil count: ≤400 eosinophils/ml (low) vs. >400 eosinophils/ml (elevated). Baseline characteristics: biomarkers, demographics, comorbidities and markers of disease severity. Univariable logistic regression was used to identify baseline characteristics associated with >400 eosinophils/ml (p≤0.05). These characteristics were then included in a multivariable model and reduced to a list of noncollinear factors. Results: Of 130,248 patients, 16% had >400 eosinophils/ml. Thirtynine percent of the patients with ≤400 eosinophils/ml and 31% with >400 were male. Median age (Interquartile range) was 45 (31-61) and 50 (37-63) for ≤400 and >400 eosinophils/ml, respectively. Table shows baseline risk factors associated with >400 eosinophils/ml (Reference category=1.00). Conclusion: In this study, several clinical and demographic features were predictive of elevated blood eosinophil counts in asthma patients. These data could be used to stratify patients who could benefit from more intensive and/or targeted asthma therapies, potentially improving control and reducing costs. Baseline risk factors associated with >400 blood eosinophils/ml (Reference category=1.00) A. DeWaters *1 , M.P. Henao 2 , E. Rael 2 , 1. Hummelstown, PA; 2. Hershey, PA. Rationale: Gastroesophageal reflux disease and asthma are both among the most common diseases treated in the outpatient setting. Studies suggest that asthma and gastroesophageal reflux disease are comorbid conditions and control of gastroesophageal reflux disease may lead to improvement of asthma symptoms. Therefore, it was hypothesized that the presence of gastroesophageal reflux disease would be associated with less control of asthma symptoms. Here we report the correlation between the presence of gastroesophageal reflux disease and the severity of asthma symptoms, as measured by the Asthma Control Questionnaire. Methods: After informed consent and IRB approval, 389 asthmatics recruited to the study between 2011 and 2014 underwent clinical assessment including review of the ACQ quality of life, and assessment of gastroesophageal reflux disease. The independent sample t-test divided the cohort by ACQ scores into group 1 (ACQ < 1.50) and group 2 (ACQ > 1.50). Results: 63 percent of 257 asthmatics in group 1 reported having a diagnosis of gastroesophageal reflux disease versus 65 percent of group 2 subjects. Group 1 asthmatics with gastroesophageal reflux disease had an average ACQ score of 0.63, whereas group 1 asthmatics without gastroesophageal reflux disease had an average ACQ score of 0.56. Group 2 asthmatics with and without gastroesophageal reflux disease had an identical average ACQ score of 2.6. Conclusion: Among asthmatics with well-controlled symptoms, the presence of gastroesophageal reflux disease resulted in a slightly increased ACQ score. However, among asthmatics with uncontrolled symptoms, the presence of gastroesophageal reflux disease did not result in any increase in the ACQ score. There was no statistically significant correlation between the presence of gastroesophageal reflux disease and more severe asthma symptoms in patients with uncontrolled asthma. Rationale: Emergency room visits and hospitalizations due to acute asthma exacerbations result in substantial healthcare costs and loss of productivity in the workforce. Although hospitalizations and deaths due to asthma have steadily declined since the 1980s, asthma prevalence is increasing. Obesity is also on the rise, and several studies have shown that obesity is a risk factor for asthma and poor asthma control. The Asthma Control Questionnaire (ACQ) is a vali-dated instrument for measuring asthma control in adults, with a score greater than 1.5 predicting a higher risk of visiting an emergency department in the subsequent two weeks. We hypothesized that in a cohort of asthmatics at a university-based allergy, asthma, and immunology specialty clinic, body mass index (BMI) would correlate with ACQ score. Methods: After informed consent and IRB approval, 356 asthmatics recruited to the study between 2011 and 2014 underwent clinical assessment including review of BMI and ACQ-7 scores. The independent sample t-test divided the cohort by ACQ scores into group 1 (ACQ < 1.5) and group 2 (ACQ > 1.5). Results: Groups 1 and 2 had 236 subjects and 120 subjects, respectively. The average ACQ score for group 1 was 0.6, and for group 2 was 2.6. Group 1 had a lower average BMI (27.1, 95% CI 26.2 -28.0) than Group 2 (29.9, 95% CI 28.2 -31.5). Conclusions: Asthmatics with ACQ scores less than 1.5 had a lower BMI than asthmatics with ACQ scores greater than 1.5. This finding contributes to the growing body of evidence showing a link between BMI and asthma. Further research is needed to elucidate this relationship. Introduction: The NHLBI recommends ongoing assessment of asthma control using a validated self-assessment questionnaire such as the Asthma Control Test™ (ACT). Variable use and poor electronic medical record (EMR) documentation of the ACT prompted an organization-wide quality improvement project. Methods: Use of the ACT has been advocated for all children ≥4 years presenting with asthma to clinics within this tertiary care children's hospital. In 2011, a process was initiated to facilitate consistent completion at outpatient visits and subsequent documentation in the EMR, with an iPad version of the ACT created in 2012. Electronic data reports guided monthly assessment of barriers. Plan-Do-Study-Act (PDSA) cycles facilitated local improvement. Interventions were spread from the pilot clinic to a total of seven clinics in 2013. Data was analyzed using Excel QI Macros. The Office of Research Integrity determined that this project does not involve human subjects research. Results: In 2011, age appropriate paper questionnaires were programmed to print out with registration paperwork for patient/caregiver completion. Providers manually entered calculated ACT scores into EMR-based Asthma Action Plans. In September 2012, a pilot clinic began patient/caregiver completion of the ACT on iPads with the score auto-populating the EMR. This clinic increased ACT documentation from an average of 17% to 75% by May 2014, with one month as high as 92%. Iterative PDSA cycles focused on reducing variation and embedding process changes into daily activities. Survey results uncovered barriers. Provider support tools, such as pictorial EMR guidance, were created. Ongoing refinement of electronic reports optimized the approach for identifying the appropriate patients for the iPad-based ACT. Sharing incremental results fostered competition that drove improvement. The ACT was also set up on each exam room computer in case of a lapse in iPad connectivity. Once these processes spread to six more clinics, the aggregate EMR documentation by all clinics improved to 62% by May 2014. Conclusion: Facilitating PDSA cycles aimed at addressing obstacles and adapting processes can improve completion and documentation of the ACT. The goal of 75% was achieved and sustained for 12 months in the pilot clinic, but the overall goal of 90% for all clinics may depend on more direct engagement of providers. The pilot clinic increased EMR documentation of the ACT from an average of 17% to 45% by March 2013, but with wide variation in results. Iterative PDSA cycles focused at reducing variation and embedding the process changes into daily activities resulted in improvement to an average of 75% by May 2014. Introduction: Asthma guidelines recommend controller medications for persistent asthma, plus short-acting bronchodilators in case of symptoms. It has been shown a greater use of rescue medications than controllers, which associates with poor asthma control. The Instituto Mexicano del Seguro Social (IMSS) provides medical care to 58 million people and has a group of medications for asthma treatment. Objective: To know the trend of the use of controller, rescue and emergency medications for asthma during a 6-year period. Methods: The use of controller (fluticasone, salmeterol/fluticasone, budesonide/formoterol, beclomethasone, montelukast, zafirlukast), rescue (salbutamol, salbutamol/ipratropium) and emergency (aminophyline, nebulized: salbutamol, salbutamol/ipratropium, beclomethasone and budesonide) medications for asthma was analyzed according to the nationwide registries of the IMSS from 2007 to 2012. Results: In the analyzed period, the rescue medications increased from 2,537,882 to 3,205,101 units (26.3%), and controllers from 1,702,606 to 3,565,548 units (109.4%). The controllers with greater increment were: salmeterol/fluticasone (702%), montelukast (79%) and beclomethasone (77%). Between 2010 and 2011 the trend in the use of controllers was greater than that of rescue medications. In the emergency medications there was a 56.8% decrease in the use of aminophyline, and a 320% and 244% increase in the use of salbutamol/ipratropium and budesonide, respectively. In 2009 we developed and communicated our institutional asthma clinical practice guidelines. Conclusion. Although both types of medications for chronic asthma increased within the studied period, the trend in the use of controllers greatly surpassed the increase in rescue drugs. As to the emergency medications, the antiinflammatory drugs revealed an increased tendency. Spreading of the institutional asthma guidelines might partially explain these trends. Research Objective: Pediatric asthma affects 10% of children age 0-10 and 15% of middle and high school students and is a significant contributor to morbidity in children covered by public insurance. To evaluate the impact of interventions, or to design interventions to have the greatest impact with limited resources, it is useful to understand the status quo within a system. This study uses retrospective Medicaid claims data to develop and quantify a set of measures around pediatric asthma in Georgia geographically and over time. Methods and Data: The baseline measures were calculated using Medicaid MAX claims data for children age 4-17 on Medicaid in Georgia and North Carolina from 2005-2009. We quantify asthma-related visits by race, age group, location, provider type, and the costs of those services. We also identify patients with persistent asthma and develop measures around medication adherence and the use of long term controller medication. We use ICD-9-CM codes to extract the relevant data, and we generate measures within areas such as counties and by subpopulations. Results: The findings include a set of baseline measures for pediatric asthma in Georgia and North Carolina 1) overall, 2) by geographical area, and 3) over time. For utilization, we find the highest percentage of patients with an asthma diagnosis among blacks but the highest number of visits in the population that is non-black and non-white. The number of patients per 1000 children decreases with age but the cost per visit increases slightly with age. On treatment measures, we find no differences with respect to age but statistically lower adherence to medication among the black patients. Measures of utilization, cost, and treatment varied significantly across the state, with a few areas identified as extreme on each. Some results varied over time. Conclusions: Significant variations in pediatric asthma measures exist geographically within Georgia and North Carolina, and some measures show changes over time. The cost measures tend to be higher in some urban and rural areas and may be driven by different underlying factors. Introduction: Asthma camp allows children with asthma to participate in camp activities while providing medical support to ensure a safe environment for their camp experience. There is minimal data on the ability of asthma camp to increase asthma knowledge in children with asthma. This study evaluated the effectiveness of a local asthma camp in increasing asthma knowledge in children ages 7-17 who have asthma. Methods: The Asthma Self-Management Questionnaire (ASMQ), previously validated in adults, was given to 40 children attending asthma camp. The survey was given on the first and sixth day of camp. IRB approval and informed consent was obtained from all research participants. Statistical significance was determined by a paired-samples t-test. Results: Topics covered in the curriculum during asthma camp included the definition of asthma, proper inhaler use, asthma symptoms and triggers, and the difference between controller and rescue medications. Initial analysis of the full ASMQ demonstrated no significant change between pre-and post-camp scores (p = 0.14). Further analysis of the questions from the ASMQ that were not covered in the curriculum during asthma camp was performed. There was no significant change between pre-and post-camp scores (p = 0.34). Analysis of the questions from the ASMQ that were covered in the curriculum during asthma camp demonstrated a significant increase in the scores between preand post-camp t(39) = 2.32, p < 0.05, with a mean score increase of 0.53, 95% CI for difference = 0.07 to 0.98. Conclusion: With improvement between preand post-camp test scores, our data suggests that asthma camp can improve short-term asthma knowledge in asthmatic children attending camp. It is not possible to draw conclusions regarding which aspect of asthma camp led to the observed improvement. Asthma camp may provide opportunities for children to understand their disease better. Caution should be used when interpreting these results as the ASMQ has not been validated in children. Further studies are needed to investigate other potential benefits that could be provided by attending asthma camp. Introduction: A novel, inhalation-driven albuterol (AB) multidose dry powder inhaler (MDPI) has been developed that eliminates the need to coordinate actuation with inhalation. Methods: Subjects aged 12 years and older with persistent asthma were enrolled in 1 of 3 studies: two 12-week, multicenter, randomized, double-blind, repeat-dose, parallel-group studies (NCT01424813 and NCT01747629) to evaluate the efficacy and safety of AB MDPI 180 mg vs placebo MDPI (PBO) administered 4 times a day (QID) and one 52-week study (NCT01698320) that included a 12-week, double-blind phase during which subjects were randomized to receive either AB MDPI 180 mg or PBO QID followed by a 40-week, open-label phase during which all subjects received AB MDPI as needed. Safety results from the 12-week phases of all 3 studies were pooled for evaluation. IRB approval and informed consent from all subjects were obtained. Primary efficacy endpoints are reported elsewhere. Results: Overall, adverse events occurred in 50% of patients in the PBO group (n=333) and 40% of patients in the AB MDPI group (n=321) during the pooled 12-week dosing period; upper respiratory tract infections (11% PBO and 10% AB MDPI), nasopharyngitis (6% and 5%), and headache (6% and 4%) occurred most frequently. The incidences of β 2 -agonist-related events during the pooled 12-week dosing period were low (<1%) in both groups. The most frequent adverse events reported in patients treated with AB MDPI during the 40-week open-label phase were nasopharyngitis (12%), sinusitis (11%), and upper respiratory tract infection (9%). There were no clinically meaningful trends in clinical laboratory variables and vital signs from screening to week 52 in those who remained on AB MDPI for the entire study. There were no electrocardiographic findings considered clinically relevant in AB MDPI-treated subjects at any time point in the 52-week study; no subject had a QTc interval length (Bazett or Fridericia) >500 msec at any time point. Conclusions: The safety profile of AB MDPI in these studies was comparable to PBO and consistent with that of the wellcharacterized profile of AB in subjects with asthma. This study was sponsored by Teva Pharmaceuticals. Introduction: A novel, inhalation-driven, multidose dry powder inhaler (MDPI) has been developed that eliminates the need to coordinate actuation with inhalation. Methods: Data from subjects 12 years of age and older with persistent asthma who were enrolled in two 12-week, multicenter, randomized, double-blind, repeat-dose, parallel-group studies (NCT01424813 and NCT01747629) were pooled to evaluate the efficacy and safety of albuterol (AB) MDPI 180 mg compared with placebo MDPI (PBO) administered 4 times a day (QID). IRB approval and informed consent from all subjects were obtained. Safety endpoints are reported elsewhere. The primary efficacy endpoint was baseline-adjusted FEV 1 area-under-the-effect curve over 6 hours postdose (FEV 1 AUC 0-6 ) over 12 weeks. Results: AB MDPI-treated subjects (n=153) experienced significantly (p<0.0001) greater improvements in baseline-adjusted FEV 1 AUC 0-6 compared with PBO subjects (n=163) over the 12-week study period (AB MDPI, 1.20 L x hr vs PBO 0.33 L x hr; mean difference 0.87±0.10 L x hr). The mean change from baseline in FEV 1 for the pooled population is shown in the Figure. Significant (p<0.0001) increases in baseline-adjusted FEV 1 AUC 0-6 in AB MDPI-treated subjects were apparent on days 1 and 8 and maintained through day 85 compared with PBO subjects (mean differences of 1.06, 0.75, and 0.81 L x hr vs PBO for these 3 time points, respectively). Of the 153 subjects treated with AB MDPI, 110 (72%) achieved a 12% increase in FEV 1 within 30 minutes postdose on day 1 with a median time to onset of 5.2 minutes, and median duration of effect (12% increase) of @3 hours for one study and 4 hours for the other. Ninety-one AB MDPI-treated subjects (59%) achieved a 15% increase in FEV 1 within 30 minutes postdose on day 1 with a median time to onset of 5.4 minutes, and median duration of effect (15% increase) of @2 hours for one study and 3 hours for the other. Conclusions: AB MDPI was consistently superior to PBO over the 12-week treatment period on spirometry-based measures. These data confirm that during periods of reg-ular usage over 12 weeks, AB MDPI continued to produce clinically significant bronchodilation compared with PBO. This study was sponsored by Teva Pharmaceuticals. Asthma and obesity are chronic disorders, and are increased in prevalence among young people. It is proposed that obesity and overweight has an independent effect on lung function in asthmatic patients. We barely know longterm obesity effects in this patients. Methodology: The study was conducted in our Hospital. The study group where obese or overweight with or without asthma. It was performed spirometry with reversibility in a baseline measurement and after 5 years follow-up, besides weight, height and BMI (Body Mass Index). Symptoms of asthma conducted through validated questionnaires: ISAAC Questionnaire and Asthma Control Test. Results: We invited to participate 90 patients, of whom 5 decided not participate. 85 patients were included but cannot schedule appointment to 35, because change of residence and phone number. We studied a total of 50 patients, of whom 33 realize spirometer, and 17 is still pending. Institutional Review Board approval and informed consent was obtained from all research subjects.Of the 50 patients studied, 22 were women and 28 men, the average age, after 5 years of follow-up for both groups was 18.7. Within the studied groups compared with the 5-year follow-up, significant difference was observed: in weight (p= 0.000), height (p= 0.000) and BMI (p= 0.000). In spirometric values, the comparison group at baseline and follow-up, significant differences was found in forced vital capacity (p= 0.000), forced expiratory volume in one second (FEV1 p= 0.000), after bronchodilator FEV1 (p= 0.004), reversibility (p= 0.009), (PEF expiratory flow p=0.001).Of the 50 patients evaluated, at the beginning of the follow up 12 patients (24%) had asthma. There were 6 patients who were monitoring newly diagnosed asthma (33%). Of the 18 asthmatic patients in total, we classify according to symptoms, through the ACT questionnaire, 5 patients were in poor control, 7 with partly controlled asthma and 6 with well-controlled asthma. Initial asthmatic patients significant differences were found in weight and in spirometric values in FVC, FEV1, and PEF (Table no. 1). Conclusion: In this study we found that obese patients with asthma, they referred to be controlled and partially controlled. We found that obese asthma patients remained with better spirometric values compared to baseline values. In patients with newly found diagnosis were in ranges of overweight and obesity. Introduction: Albuterol (AB) delivered via a novel, inhalation-driven, multidose dry powder inhaler (MDPI) eliminates the need to coordinate actuation with inhalation. In a separate study, treatment of adolescents and adults with persistent asthma with AB MDPI was well tolerated and demonstrated efficacy significantly greater than placebo and comparable to AB hydrofluoroalkane metered dose inhaler (HFA) at the intended doses of 90 and 180 mg. Methods: This multicenter, randomized, double-blind, double-dummy, cumulative-dose, 2-period crossover study (NCT01056159) evaluated the efficacy, pharmacokinetics (PK), extrapulmonary pharmacodynamics (PD), and safety of cumulative doses (1440 mg) of AB via MDPI or HFA to subjects (aged 18-45 years; N=47) with asthma. IRB approval and informed consent from all subjects were obtained. Subjects were treated with AB MDPI or AB HFA (90 mg/inhalation) and respective placebo in a randomized-sequence, two-period crossover. At each treatment visit, study drug was administered by cumulative dosing of 1+1+2+4+8 inhalations (separated by 30 minutes) from each device. Treatment visits were separated by 3 to 14 days of washout. The primary efficacy endpoint was baseline-adjusted FEV 1 at 30 minutes after each of the cumulative doses. Results: Thirty-eight subjects received both AB MDPI and AB HFA and experienced increases in FEV 1 over baseline at each cumulative dose level. The differences between treatments in FEV 1 at 30 minutes ranged from −0.07 to −0.03 L and were not clinically meaningful. Mean systemic exposure over 14 hours was comparable (AUC 0-t, 23,227 for AB MDPI and 20,939 for AB HFA), C max was higher for AB MDPI (4422.8 pg/mL) vs AB HFA (3303.8 pg/mL), and T max occurred earlier with AB MDPI (2.48 h) vs AB HFA (3.05 h). Extrapulmonary PD differences between treatments were not considered clinically meaningful. Adverse events reported by ≥5% of subjects treated with AB (cumulative dose of 1440 mg) included tremor (17% AB MDPI and 13% AB HFA), palpitations (9 and 2), and headache (9 and 4). Conclusions: The overall efficacy, PK, PD, and safety profile of AB MDPI and AB HFA were generally similar. The results of this and previous AB MDPI studies support evaluation of a dose of 180 mg in adults with asthma. This study was sponsored by Teva Pharmaceuticals. onset asthma was diagnosed when first episodes of symptoms occurred before 40 year old and late onset one after 40 according to GINA 2012 guidelines. The control group included 285 non-atopic volunteers. Single nucleotide polymorphisms of CD14 C-159T were detected by PCR. Patients and volunteers were recruited at the Crimean State Medical University, Simferopol, Crime, and provided written informed consent for the genetic study. Results: In earlyonset asthma patients CC genotype was detected in 74 patients, CT in 142 and TT in 46; in late-onset asthma: CC in 31, CT in 27, and TT in 11; while in the control group CC in 97, CT in 146, and TT in 42. The allele frequencies were 55 % (n=290) for the C allele and 45 % (n=234) for the T allele in the earlyonset asthma c (OR, 1.19; 95% CI, 0.94-1.52; P=0.15); 64% (n=89) for the C allele and 36% (n=49) for the T allele in the late-onset asthma (OR, 0.81; 95% CI, 0.55-1.99; P=0.29); and 60% (n=340) for the C allele and 40% (n=230) for the T allele in the controls. Conclusion: CD14-C159T polymorphism is not associated with adult early and late-onset asthma in Crimean population. The polymorphism in this population did not differ from that seen in healthy control subjects. Introduction: Allergic rhinitis (AR) is frequently encountered in patients with atopic asthma (AA). The influence of allergic rhinitis on clinical symptoms in asthmatics depends significantly on asthma severity. Methods: Consecutive patients 17-26 years of age with mild intermittent (MI) and mild persistent (MP) allergic asthma were enrolled in the study with informed consent obtained. Evaluation included lung function assessment, skin prick tests, broncho-provocation tests,(BPTs), bronchodilator (BDT) response, total and allergenspecific IgE levels. Results: Patients in both groups, 63 in MI and 72 in MP, had similar age(mean 19 years) and AA duration (mean 5 versus 6 years in MI and MP groups) and were equally of rural and urban origin. Prevalence of persistent AR (P-AR) was significantly greater in MP asthma (54% in MI asthma and 78% in MP asthma, p<0.05). Patients with persistent AR had on average higher rate of exacerbations independent of asthma severity: more than 1 exacerbation per month occurred in 26% of patients with intermittent AR (I-AR) or without AR versus 68% of patients with persistent AR (p<0.05) in the MI asthma group. More then 1 exacerbation per month was seen in 44% of patients with I-AR or without AR versus 86% of patients with P-AR (p<0.05) in the MP asthma group. Patients with persistent AR had much higher rates of symptoms in relation to overall dust load both in MP asthma (95% in P-AR patients versus 25% in all others, p<0,05) and MI asthma (95% in P-AR versus 68% in all others, p<0,05). In MI asthma patients presence of persistent AR was also associated with a higher mean number of relevant non-specific triggers (1.76±0.93 in P-AR versus 0.97±0,84 in all others, p<0.05), clinical reaction to cold air (27% in P-AR patients versus 3% in all others, p<0.05) and exercise-related symptoms (81% in P-AR versus 48% in all others, p<0.05). Conclusion: Allergic rhinitis has significantly higher prevalence in MP asthma. Patients with persistent allergic rhinitis have two-fold or higher rate of exacerbations independent of asthma severity. Among non-specific asthma triggers the most clinically relevant for all patients with persistent allergic rhinitis was the total dust load. The number of non-specific asthma triggers was also greater in patients with persistent allergic rhinitis in both mild intermittent and mild persistent asthma. Background: There is a relationship between atmospheric pollutants and climatic factors and visits to the emergency room for asthma, however there is lack of knowledge on the situation of this phenomenon in Mexico City. Objective. To assess the correlation of atmospheric pollutants and some climatic factors with the number of cases of asthma exacerbations in children in a third level hospital in Mexico City during the last five years. Methods: Case registries of asthma exacerbations at pediatric emergency room, stratified by age (0-23 months, 24-71 months, 6-12 years, 12-17 years), and their correlation with daily values of atmospheric pollutants (ozone, sulphur dioxide, nitrogen dioxide, carbon monoxide and PM 10 ), temperature and relative humidity, from 2009 to 2013, were analyzed. IRB/HAC approval was obtained. Results: During these five years there were 5331 visits for asthma exacerbations, with an annual average of 1066. There was a gradual decrease in cases, from 2363 in 2009 to 505 (21%) in 2013. There was a male predominance in all ages (ratio 1.7 : 1). The age groups 24-71 months and 6-12 years had the highest number of cases, 1766 (33.1%) and 1827 (34.3%), respectively. During fall there was the highest number of visits 2069 (39%), and the lowest was in spring, 801 (15%). The correlation between asthma and environmental factors was: CO: R=0.242, NO 2 : R=0.175, SO 2 : R=0.092, PM 10 : R=0.056, O 3 : R=-0.062, relative humidity R=-0.106 and temperature R=-0.36. Conclusions: A gradual and significant reduction in the number of asthma visits to the emergency room was observed during the period of five years. There was a significant increase in asthma exacerbations related to seasonal effect. There was a predominance of male pre-school and school children in relation to asthma exacerbations. The highest correlation between atmospheric pollutants and asthma cases was related to carbon monoxide. There was inverse correlation between asthma exacerbations and weather factors (humidity and temperature). Introduction: Little is known about benefits of continuous omalizumab (OMA) treatment to asthma patients in real world. We examined the lapses in asthma control in patients who continued vs. discontinued OMA treatment in a real-world setting. Methods: This retrospective cohort study used a large HIPAA-compliant commercial healthcare claims database (1/1/08 to 12/31/10). Identified patients were ≥12 years old, had ≥2 medical asthma-associated claims, filled ≥2 asthma controller medications in the identification period (1/1/09-12/31/09), and used OMA continuously for ≥6 months. After ≥6 months of OMA therapy, patients were stratified as continuers (continued OMA until dropout or 12/31/10) or early discontinuers (stopped OMA for >90 days) and followed for ≥3 months after the index date. The primary outcome was the risk of lapse in asthma control during follow-up, reported as episodes per 100 patientyears (PY), measured as any asthma-related hospitalization or emergency room visit, ≥2 oral corticosteroid (OCS) or ≥6 short-acting beta-agonists filled in 1 year. We used Cox regression models to assess risk of the outcome, adjusted for baseline patient characteristics (e.g., demographics and the number of chronic conditions). The study did not involve human subjects, thus IRB review was not needed. Results: We identified 414 continuers and 240 discontinuers among eligible patients; mean age: 46.2 years, 62.5% women, and 15.6% had COPD. At baseline, the groups were balanced by demographics and asthma control. Patients were followed for a median 415 days; the discontinuers had no OMA use after the discontinuation date. Overall, there were 32.5 episodes of lapses in asthma control per 100 PY of follow-up: 27.9 per 100 PY for continuers vs. 43.3 per 100 PY for discontinuers (p = 0.002, Table) . Discontinuers had worse individual clinical outcomes that defined lapses in asthma control than did continuers (Table) . Adjusting for patient characteristics, discontinuers had a significantly higher risk of having lapses in asthma control than continuers (adjusted hazard ratio 1.66, p<.001). Conclusions: Real-world evidence suggests that asthma patients who receive continuous (>6 months) OMA treatment may have fewer lapses in asthma control than those who prematurely discontinue OMA after 6 months. The genus Rhodiola is important herb used for thousands of years in Traditional Chinese Medicine for treating various kinds of diseases, including inflammation-related diseases such as pneumonia, chronic bronchitis, asthma, and rheumatoid arthritis. Salidroside (p-hydroxyphenethyl-beta-d-glucoside, C14H20O7, molecular weight 300.30), which is one of the most potent ingredients of the genus Rhodiola, has been reported to have a broad spectrum of pharmacological properties. Recent study indicates an anti-inflammatory role for salidroside in lipopolysaccharide (LPS)-induced acute lung injury (ALI), but the mechanisms of anti-inflammatory action are not clear. In the present study, anti-inflammatory activity of salidroside was analyzed in vitro using LPS-induced inflammatory reactions in rat alveolar macrophages. Inflammatory mediators such as TNF-α, IL-6, and NO were measured by the Griess reaction for NO and Enzymelinked immunosorbent assay (ELISA) for other inflammatory mediators. QRT-PCR was used to measure the mRNA expression of TNF-α, IL-6, and iNOS, and the protein levels of iNOS, p65, p-p65, IκB-α, and p-IκB-α were analyzed by western blot. EMSAs and reporter gene assays were used to evaluate DNA binding and transcriptional activities of NF-κB. ChIP analysis was used to detect the binding of p65 to the iNOS promoter. NF-κB nuclear translocation was analyzed by western blot. Salidroside significantly inhibited LPS-stimulated production of NO by suppressing the expression of iNOS. EMSA demonstrated that LPS treatment increased p65-DNA binding, whereas this interaction was decreased by salidroside. ChIP analysis revealed that salidroside markedly inhibited LPS-induced p65 binding to the iNOS promoter. Western blotting confirmed that salidroside effectively inhibited the nuclear translocation of p65. We also examined the effect of salidroside on the induced degradation and phosphorylation of IκBα protein by LPS. The results showed that salidroside could change the kinetics of IκBα phosphorylation and degradation. Salidroside also suppressed TNF-and IL-6 at the mRNA level, and then reduced the release of TNF-α and IL-6 induced by LPS. In conclusion, our results suggest that salidroside can block LPS stimulated inflammatory responses, and may be used as a potential anti-inflammatory agent for the prevention and treatment of inflammation-related diseases. Immune response and pathologic events in allergic asthma are regulated by specific T cell subsets and the cytokines that they secrete. Mast cells and eosinophils are key effectors in the cascade of airway inflammatory events which induce bronchoconstriction, influx of inflammatory cells and airway remodeling. KIT, the receptor for Stem Cell Factor (SCF), is expressed on mast cells and eosinophils in the lung, and for that reason, we are examining inhibition of SCF/KIT signaling as a possible therapeutic in allergy and inflammation. KTN0158 is a humanized IgG1 monoclonal antibody that inhibits signaling through KIT. This antibody specifically binds the Ig-like domain 4 of KIT, and blocks SCF binding and receptor dimerization and activation. In vitro studies demonstrate that KTN0158 is a potent inhibitor of SCF-dependent KIT activation and of degranulation enhanced by SCF in the human LAD2 mast cell line. KTN0158 has similar binding affinity for human, monkey, dog and cat KIT, but does not cross-react with murine KIT. A placebo-controlled, cross-over design pilot study was conducted to evaluate the ability of KTN0158 to reduce airway eosinophilia and airway reactivity in experimentally asthmatic cats sensitized with Bermuda Grass Antigen (BGA). Cats received 20 mg/kg KTN0158 or placebo on days -1 and 14, and airway reactivity was assessed using BGA or methacholine bronchoprovocation (day 0 or day 28) and ventilator-acquired pulmonary mechanics, as well as bronchoalveolar lavage to assess airway inflammation. Cats treated with KTN0158 had significant reduction in airway eosinophilia during the early-(p=0.009) and late-phase (p=0.032) asthmatic reactions, suggesting further investigation of KIT inhibition with KTN0158 in allergic asthma is warranted. To this end, studies to investigate the potential benefit of KTN0158 in mast cell-related diseases and to evaluate safety are ongoing. R. Anand *1 , E. George 2 , M. Yu 2 , J.L. Ambrus 2 , L. Shen 2 , 1. Getzville, NY; 2. Buffalo, NY. Introduction: There is a high incidence of secondary lymphomas in patients with autoimmune diseases, especially Sjogren's syndrome (SS). We have developed an animal model, the IL-14alpha transgenic mouse (IL14aTG) that reproduces all the features of SS seen in patients in the same relative time frame, including the development of lymphoma. We have evaluated genetic changes in the spleens of these mice during different stages of the disease to determine when genes characteristic of SS lymphoma are aberrantly expressed in the animals with the disease. Methods: QPCR was performed on the splenic RNA of IL14aTG mice and control C57BL/6 mice for bcl-6 and bcl-10 at 6, 10 and 14 months of age. In this animal model, disease involving the salivary and lacrimal glands starts at 6 months of age while systemic disease starts at 10 months of age. Some mice will start developing lymphoma at 14 months of age while the majority of mice develop lymphoma only by 18-20 months of age. Results: In IL14aTG mice but not C57BL/6 mice, expression of bcl-6 and bcl-10 decreased at 14 months of age, but not at earlier time points. None of the mice with these genetic changes had yet developed lymphoma. A. Gharib *1 , A. Gupta Louis 2 , S. Agrawal 2 , S. Gupta 2 , 1. Los Angeles, CA; 2. Irvine, CA. Background: Detailed immunological analyses have not been reported in recently recognized Non-tuberculous mycobacterial infection of the skin. The main purpose of this investigation is to present extensive immunological analysis of a patient with cutaneous non-tuberculous mycobacterial infection (mycobacterium avium complex). Material and Methods: Peripheral blood from the patient and healthy controls were analyzed for CD3+, CD4+, CD8+ T cells, regulatory T cells (FoxP3+CD4+CD25+), naïve, transitional, marginal zone, IgM memory, switched memory B cells and plasmablasts, expression of TACI and BAFF-R on naive and memory B cells, expression of IFN-γ receptor and IL-12 receptor on lymphocytes and monocytes, NK cell phenotype and cytoxicity against K562 target cells using various effector:target ratio. Lymphocyte functions were measured by in vitro response to mitogens (PHA, ConA, PWM) and recall antigens (mumps, candidate, PPD, and tetanus toxoid) by 3 H incorporation. Toll-like receptors (TLR) were analyzed on monocytes. Results: Analysis of lymphocyte subsets showed severe T cell lymphopenia of CD3+, CD4+ and CD8+ T cells) and an impaired response to mitogens and antigens. T regulatory cells were significantly reduced. Analysis of B cells revealed low serum IgM and an increased transitional B cells with normal marginal zone, IgM memory and class switch memory B cells, and marginal zone B cells. Expression of TACI and BAFF receptor on both naiive and memory B cells, and expression of and IFN -γ receptor and IL-12 receptor on lymphocytes and monocytes were normal. TLR4 expression was increased, whereas the expression of TLR5, TLR7, and TLR9 was decreased. NK activity was found to be decreased. Conclusion: This patient illustrates that cutaneous non-tuberculous mycobacterium infections may be associated with severe immune deficiency including T cell lymphopenia, impaired cell mediated immunity, deficiency of T regulatory cells, alterations in transitional B cells, selective IgM deficiency, altered TLR expression, and an impaired NK cell cytotoxicity. L. Helfner * , A.M. Jongco, Great Neck, NY. Introduction: Complement component deficiencies are rare, and predispose to autoimmunity or increased susceptibility to recurrent infections with encapsulated bacteria. The types of infection relate to the function of the missing components. For example, terminal complement component deficiency frequently presents with Neisseria species infections. Methods: We describe an 11-month old male who presented originally with lethargy and fever that developed into GAS sepsis with toxic shock syndrome. He received a 10-day course of IV antibiotics and was discharged on oral cephalexin. Shortly after discharge, he was re-admitted with fever and found to have extended-spectrum beta lactamase producing (ESBL) E. coli urosepsis, which responded well to a 10-day course of IV ertapenem. For UTI prophylaxis, the patient now receives trimethoprim-sulfamethoxazole daily, and has done well following his second hospitalization. He is currently receiving routine vaccinations as per CDC guidelines. Results: Laboratory evaluation was remarkable for undetectable CH50 on two separate occasions, as well as mild T cell lymphocytosis (CD3+ of 8326/uL (reference range 1700-3600), CD4+ of 6285/uL (range 1700-2800), and CD8+ of 1654/uL (reference of 800-1200)). B and NK cells were unremarkable for age. Lymphocyte proliferation to pokeweed and phytohemagglutinin was normal. G6PD was mildly depressed at 3.5 U/g Hb (range 4.6-13.5). Myeloperoxidase stain was normal. HIV serologies were negative, and he had detectable hepatitis B surface antibody. Total IgE was elevated at 323 IU/mL (range 0-15), with normal IgA, IgM, and IgG for age. Levels of C2, C3, C4, C5, C7, C9, C1q, mannose binding lectin and AH50 were normal. C8 was decreased at 20 ug/mL (range 33-58). Further investigation of other complement components is underway. Conclusion: This patient had mildly decreased G6PD and C8 with undetectable CH50 but normal AH50, suggesting that other complement components are deficient in quantity and/or function, likely upstream of the membrane attack complex. Physicians must remain vigilant and include complement deficiency in the differential diagnosis. These patients should receive routine vaccinations, especially against diseases for which they are at increased risk. The airway smooth muscle cells (ASMC) hyperplasia is a central feature of airway remodeling. ASMC mitogenesis is promoted by several mediators such as epidermal growth factor (EGF) and tumor necrosis factor-α (TNF-α), which are countered by activators of guanylyl cyclases (GC)-cGMP pathway. This study evaluated the effect of sodium nitroprusside (SNP), an oxide nitric (NO) donator, and natriuretic peptides (ANP, CNP) on rat ASMC proliferation. Cells were isolated from tracheal fragments and were characterized using immunochemistry with specific antibody anti-α smooth muscle actin, indicating that after 3rd passage were 100% positive. Proliferation assays were performed by MTS-PMS method. Viability was confirmed by trypan blue exclusion method. To determined GC enzyme activity, cells were pre-incubated with 100 M IBMX (PDEs inhibitor) and cGMP was measured using Amersham kit. SNP (10 -5 M; n=6), ANP and CNP (n=3) in a doses-dependent manner inhibited ASMC proliferation induced by fetal bovine serum (FBS), EGF and TNFα (p <0.05). ODQ (sGC inhibitor) reversed the action of SNP (p <0.05, n=3) and improved basal proliferation and mitogenesis induced by FBS (p <0.05, n=3). We found increased of cGMP levels in response to SNP, ANP and CNP. cGMP synthesis induced by SNP, but not by natriuretic peptides, was inhibited by 100nM ODQ (p <0.05, n=3). NO, ANP and CNP increase cGMP levels by different transduction systems and inhibit ASMC proliferation. Moreover, basal activity of sGC regulates ASMC mitogenesis. This signaling pathway might be altered in airway remodeling and represent an important therapeutic target to ASMC hyperplasia. Background: Cytidine deaminase (CDA) and adenosine deaminase (ADA) basal serum activity may be impacted by acute and chronic infection states. Methods: Two groups formed on a wide (n = 10, male and female, 24-31 age) and narrow (n = 19, only male, 18-25 age) principle of sampling as controls were compared to infectious mononucleosis (IM) patients (n = 10, male and female, 18-34 age), chronic HCV infection patients (n=29, male and female, 27-70 age), and patients with pneumonia developing in the 2012-13 influenza epidemic season (n=10, male and female, 6-16 age) by assessing CDA and ADA activity (indophenol colorimetric test), CD19 + cells content (flow cytometry), B-cells clonal status (immunoglobulin heavy chains rearrangement) and PCR analysis using ISH assay (InVivoscribe, USA) and GelAnalyzer 2010a soft. Results: CDA activity in pneumonia (8.14±2.72 U), as well as in infectious mononucleosis (4.37±2.22 U) was found to be high in comparison with the control groups: 1.1±0.4 U for the wide cohort and 2.29±0.5 U for the narrow cohort. In contrast, chronic HCV patients had no increased CDA levels (1.21±0.17 U). A polyclonal pattern was seen in both wide and narrow controls, IM and chronic HCV infection patients. Both control cohorts show similar ADA activity levels: 12.08±5.24 U for wide and 11.10±2.2 U for narrow cohort. A two-fold increase in ADA activity in IM patients (27.56±9.87 U) and the absence of significant deviations in ADA levels for chronic HVC (14.53 U) were seen. Despite the low absolute values, CDA and ADA enzymatic activity proved itself to be among the most individually variable immunologic parameters. Conclusions: CDA and ADA enzyme activity is an informative parameter with potential to be a biomarker of hidden immunodeficiency status. Introduction: Oral lichen planus (OPL) is the most common immune disease of oral mucosa, having an increased risk of malignancy. Cytokines (IL-1, TNF-α, TGF-β) in blood may impact disease progression in erosive oral lichen planus. Methods: 97 patients aged 18 to 60 years were studied including 35 with erosive oral lichen planus, 32 with non-erosive oral lichen planus and 30 healthy control subjects of similar age. The levels of serum cytokines IL-1, TNF-α, TGF-β were determined by enzyme-linked immunosorbent assay ("Immunotech"; France). Results: During relapse of erosive oral lichen planus there is change in select cytokines compared with control group levels including IL-1 (0.6±1.5 pg/ml) being 2-fold increased versus the healthy controls and TNF-α being 4.3-fold increased (0.3±1.6 pg/ml versus 1.3±1.2 pg/ml). TGF-β ( 3.2±1.2 pg/ml) however is reduced 3.8-fold versus healthy controls (12.3±1.1 pg/ml). In erosive chronic OLP there is an increase in IL-1 by 1.8fold, an increase of TNF-α by 1.8-fold and an increase of TGF-β (29.6±2.2 pg/ml) compared to healthy controls (12.3±1.1 pg/ml). In chronic OLP IL-1 indexes were increased 3.7-fold in comparison to OLP relapse and TNF-α increased 7.7-fold and TGF-β increased 9.2 -fold compared to disease relapse(p<0.05). Conclusions: Acute relapse and chronic stages of lichen planus of the oral mucosa are characterized by immunological changes detectable in peripheral blood serum including increases in concentrations of IL-1, TNF -α, TGF-β, characteristic of development of a recurrent inflammatory immune response and chronic persistent erosive disease. A. Hancharou 1 , N. Antonevich 1 , V. Chekan 1 , L.M. DuBuske *2 , 1. Minsk, Belarus; 2. Gardner, MA. Introduction: Nasal mucosa contains olfactory epithelium-derived ectomesenchymal stem cells (hOE-EMSC), which are similar to mesenchymal stem cells derived from bone marrow and adipose tissue and which may have immunosuppressive properties. Methods: Nasal mucosa samples were taken from 4 patients with non-inflammatory diseases of the nasal cavity. Explant culture methods were used to obtain hOE-EMSC cells. Cells were assayed for phenotype (CD90, CD105, CD45, CD273, CD274, CD80, CD86, CD40, nestin). Culture media from hOE-EMSC were collected and used in further experiments. PBMC isolated from donor blood were primed with CFSE, cocultured with hOE-EMSC for 3 days plus PHA, and then assayed for proliferation. PBMC were also cultured without hOE-EMSC with the addition of PHA (positive control), and with the addition of hOE-EMSC conditioned media. Results: Cells obtained were CD90 + CD105 + nestin + CD45 -. The ability of hOE-EMSC to inhibit mitogen-induced proliferation of CD4+ T cells was shown. The immunosuppressive effect did not depend on soluble factors produced by stem cells, including interleukin-10. Direct contact of hOE-EMSC with lymphocytes was required to exhibit immunosuppressive properties. HOE-EMSC expressed the co-inhibitory molecules CD273 and CD274 but were negative for CD80 and CD86. Immunosuppressive properties were dependent on CD273 and CD274 expression. Conclusion: The hOE-EMSC possesses immunosuppressive properties and may possibly be used in cellular immunotherapy of immune system disorders including autoimmune diseases and could possibly assist in prevention of organ and tissue rejection. A. Hancharou 1 , G. Davidovich 1 , I. Ramanava 1 , E. Duzh 1 , L.M. DuBuske *2 , 1. Minsk, Belarus; 2. Gardner, MA. Introduction: The content of minor leukocyte subsets in the patients with infectious mononucleosis (IM) may be altered including monocyte, dendritic cell (DC) and myeloid-derived suppressor cells (MDSC). Methods: 12 patients with acute IM, confirmed by blood serum and saliva PCR analysis, were selected for investigation. Whole blood from 10 healthy volunteers matched by age and sex was used as a control. Relative and absolute counts of leukocyte subsets were assayed using FACSCanto II cytometer: CD1c + and CD141 + myeloid DC, plasmacytoid DC, CD14 + /CD16 + blood monocytes and monocytic (M-MDSC) and granulocytic (CD15 + and CD33 + ) G-MDSC. Results: In patients with IM statistically significant myeloid DC subset redistribution was seen with increase of CD141 + cells (CD1c/CD141 index in patients: 1.9(1.4-2.6), versus in controls: 6.2(5.6-7.3), =0.00013), suggesting a role in the pathogenesis IM. Decrease of plasmacytoid dendritic cell counts in IM was observed (patients: 0.04(0.02-0.11)% versus controls: 0.4(0.250-0.520)%, p=0,000001; patients: 0.003(0.002-0.01)×10 6 /ml versus controls: 0.022(0.013-0.032)×10 6 /ml, p=0.00017), suggesting migration to lymphoid tissue or selective depletion. Monocytic myeloid-derived suppressor cell counts were reduced in acute IM (patients: 0.075(0.05-0.135)% versus controls: 0.32(0.23-0.37)%, p=0.011), explaining EBV-induced immune system hyper-stimulation and increase of HLA-DR expression on virtually all APC and T-cells. Increase of absolute (p=0.052) and relative (p=0.09) CD15 + G-MDSC counts were seen in IM but no statistically significant differences in the number of CD33 + G-MDSC cells. Significant variability of content of G-MDSC and "intermediate" and "nonclassical" monocyte subsets were noted such as in IM with CD15 + G-MDSC minimum of 0.01% and maximum of 0.22% versus controls minimum of 0.022% and maximum of 0.167%. Conclusion: Significant changes in the number of minor leukocyte subsets in blood are seen in IM. These changes may explain some of the clinical immune manifestations of IM. A. Khojah *1 , A. Bukhari 1 , O. Alpan 2 , 1. Chicago, IL; 2. Vienna, VA. Rationale: Despite the growing use of flow cytometry in evaluation of various immunodeficiency disorders, there are limited number of studies that discuss the gender effect on the reference range of different B cell subsets, especially in the pediatric age group. The aim of this study was to evaluate the effect of gender on B cell maturation in different age groups. Methods: Total of 988 subjects were included in this study. We excluded all adults whose age is 22 or older, all subjects with known primary or secondary immunodeficiency and patients with incomplete work up. All Subjects had normal evaluation including CBC, serum immunoglobulin, antibody response to vaccine especially pneumococcal titers (for subjects more than 2 years). Clinical and laboratory data including B cell subset were obtained from patients' records. Subjects were divided into seven groups based on their age. Using T test, we compared between males and females in each age group in terms of different B cell sub-populations. Data was analyzed using SPSS, version 22. Results: Both, the absolute B cell count and the percentage of B cell, drop significantly in the first 7 years of life. That drop was equal in the male and female groups. The mean of CD5+ B cell was 4 to10 % higher in males versus females in all age categories except in subjects between 19 and 21 years of age. This difference reaches statistical significance in the age groups between 13 and18 years, which might suggest a possible, but not previously described, hormonal effect on the B cell maturation. Overall, females have higher mean of switched B cell (IgG+, IgA+) and lower mean of IgM+, IgD+ B cell. Yet, these differences are more significant between 10 to18 years of age. Conclusion: These graphs highlight the difference between males and females in terms of B cell sub-populations, and the importance of having gender-specific reference ranges especially in the adolescent age group. Our results suggest a possible hormonal influence on B cell maturation. Further research is needed to investigate this theory. Introduction: CREST syndrome (Calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyle, and telangiectasia) or systemic sclerosis with limited scleroderma is very rare in children, and mainly involves skin, subcutaneous tissues, muscle and bones, and does not involve internal organs. The usual onset of scleroderma is approximately 30-65 years of age. Its incidence in children is one per one million. Case Description: An 8 year old African American girl with past medical history significant for being born at 28 weeks GA, via C-section due to abruptio placenta and pre-eclampcia, intubated right after delivery, required 3 months of NICU care, with multiple blood transfusions, PDA closed with indomethacin, pubarche at 5 years of age, with negative work up, who presented with over all 10 months history of non-pruritic, off white, rashes on elbows, knees that over the course of few months had turned hard and looked as if they were warts and finally calcified over the next few months . Also 10 months history of "icy cold" fingers and toes with bluish discoloration. On physical examination there were multiple calcified nodules on extensor surfaces of the elbows, finger tips with protruding calcified papules, sclerodactily of fingers, and telangiectasia along with bluish discoloration of finger tips to the level of PIPs. Biopsy of the lesions confirmed calcinosis cutis. Laboratory investigation including CBC, ESR, CMP, ANA with reflex remained inconclusive. Chest x-ray did not indicate any pathology. Treatment: She was started on monthly IVIG infusion along with daily PO Prednisolone, weekly PO Methotrexate and daily folic acid. Her symptoms greatly improved over 4-5 month, calcinosis of the finger tips resolved, while the rate of eruption of the new calcified lesions slowed .and resolved. Discussion: CREST syndrome is a very rare disease in children, our patient had 4 out of 5 findings and improved after outlined therapy. Treatment for scleroderma needs to be started promptly to avoid future complications. In general prognosis of sclerosis in children is better than in adults. Rationale:Intravenous gammaglobulin(IVIG) administration for immunodeficiency disease is rarely associated with a delayed serious, adverse reaction of aseptic meningitis.This condition is usually related to high doses of IVIG (1-2gm/kg).The pathogenesis is postulated to be an osmotic shift caused by small amounts of IVIG penetrating the cerebrospinal fluid and/or antineutrophil antibodies in some IVIG products activating neutrophils in the central nervous system.(1) Methods :Case Report We present a 36 year old white female with 1) selective antibody deficiency and hypogammaglobulinemia 2)car-diomyopathy with implanted pacemaker (2009)and recurrent pericaridi-tis3)moderate persistent asthma on Advair and Alvesco 4)depression and 5) a migraine condition admitted with aseptic meningitis after first infusion of IVIG 400mg /kg over 3 hours in a monitored setting.She develped a severe,pulsating, diffuse occipital, temporal and parietal headaches with retro orbital pain beginning 6 hours post infusion.The pain exacerbated with any motion of the head or neck,cough or bearing down and was associated with visual alterations and inability to register words or numbers.She experienced dull pain radiating down the neck to left precordial area and left shoulder and arm with fever,nausea and diarrhea 24 hours prior to admision.CT scan of the head demonstrated right maxillary sinus air fluid levels.Lumbar puncture(LP) revealed normal glucose 49mg/dl,normal protein 36mg/dl,WBC 512/mm3 and RBC 59/mm3 47%segmented neutrophils,51%mononuclear cells and 2%eosinophils with negative cerebrospinal fluid cultures and relief of symptoms consistent with aseptic meningitis . She had a history of recurrent sinusitis and admissions for "pneumonia" 2009-13 .Her original immune evaluation demonstrated IgG 651mg/dl(694-1618mg/dl),normal IgM(132mg/dl),A(298mg/dl), and protective tetanus titers but nonprotective pneumococcal titers( 13/14) despite recurrent immunizations and varicella zoster titers were nonprotective. Results:Aseptic meningitis was treated with systemic steroids and Omnicef for sinusitis. IgG replacement was subsequently changed to subcutaneous route without further complications. Conclusion Aseptic meningitis is a rare delayed adverse event following IVIG administration. Rationale: Hemophagocytic lymphohistiocytosis (HLH) is a rare clinical syndrome characterized by uncontrolled activation of lymphocytes and macrophages producing elevated levels of cytokines. A clinically challenging diagnosis to make, HLH manifests as fevers, cytopenias, hepatosplenomegaly, hyperferritinemia, elevated IL-2, hepatic failure, and neurologic symptoms. We present a case of a patient with persistently high fevers, pancytopenia, hyperferritinemia that was diagnosed as HLH. Methods: A 28 year old Native American male presented with fever, chills, myalgias, arthralgias, bilateral lower extremity edema, and a fifty-six pound unintentional weight loss in three months. Neither high dose prednisone for Adult Onset Still's disease, nor broad spectrum antibiotics for an infectious etiology improved the fever or hepatic synthetic function. Results: Ferritin was extremely elevated to 16,173 ng/mL (reference range 22-322). Serum immunoglobulins, including IgG, IgA, and IgM, were within normal limits. Concentration of sCD25 and IL-2 receptor were elevated. Fibrinogen was low and triglycerides were high. Infectious serologies and cultures including EBV and CMV were negative. Autoimmune markers were negative. White blood cell count with differential was normal. Liver biopsy showed steatohepatitis with stage 2 fibrosis. Flow cytometry showed normal phenotype. Bone marrow biopsy showed prominent hemophagocytosis consistent with hemophagocytic lymphohistiocytosis. Conclusion: Our patient met seven of the eight diagnostic criteria for HLH, including fever, splenomegaly, cytopenias, hypofibrinogenemia and hypertriglyceridemia, elevated ferritin, elevated sCD25 and hemophagocytosis in the bone marrow. Delays in this challenging diagnosis can result in high mortality rates due to multisystem organ failure. Because biopsies of affected sites may be normal or misinterpreted, repeat biopsies are sometimes necessary if clinical suspicion remains high. We suspect hepatic involvement in our patient despite the lack of hemophagocytosis on liver biopsy. Treatment algorithms include etoposide, dexamethasone, antithymocyte globulin, cyclosporine A, intravenous immunoglobulin, alemtuzumab, and hematopoietic cell transplant. In patients with persistent fever, hepatosplenomegaly and cytopenias, the challenging diagnosis of HLH should be considered for prompt therapy. A. Ali * , M. Segal, Philadelphia, PA. Introduction: Eosinophilic esophagitis (EoE) is a condition in which inflammatory cells invade the mucosa of the esophagus. Few cases have described the association of EoE with parasitic diseases. We present a case of EoE in a young man with Strongyloides infection. Methods: A 21 year old male returns from a medical mission trip to Kenya. Upon his return, he develops an eruptive rash on his abdomen that is self-limiting after 3 months. He also develops a cough and worsening dysphagia and is initially treated medically for acid reflux. Despite treatment, these symptoms worsen. He also experiences intermittent diarrhea, ranging from loose to watery bowel movements. Labs display eosinophilia (eosinophil count 600 c/mL (10.2%) (normal 500 c/mL)) and normal immunoglobulin E (IgE) level (93 kU/L). Results: After months of worsening dysphagia and cough, an esophagogastroduodenoscopy reveals mucosal changes including longitudinal furrows and circumferential folds in the entire esophagus. Pathology displays squamous mucosal eosinophilia with 57 in 1 high power field (normal <15 per high power field), seen superficially and deep with no microabcesses. A diagnosis of eosinophilic esophagitis is made. After one month of no resolution with swallowed corticosteroid solution, his allergist is prompted to pursue a parasitic work up. A strongyloides immunoglobulin G (IgG) antibody (Ab) ELISA is elevated at 1.22 units/mL (normal, <1.00). Ova and parasite stool test is negative. Treatment with ivermectin is initiated. His dysphagia resolves after anti-parasitic treatment, but his shortness of breath continues. Labs show persistent eosinophilia, and he is given a second treatment of ivermectin with resolution of respiratory symptoms. Conclusion: EoE is a condition that can lead to permanent sequelae including esophageal scarring and fibrosis. Strongyloides infection can cause a systemic eosinophilia, and in this patient the eosinophilia from the untreated Strongyloides infection was associated with esophageal infiltration of eosinophils. Strongyloides is treated with anti-parasitic agents, and can be monitored by symptom resolution, eosinophilia on labs, or repeat strongyloides Ab. Rationale: Wolf-Hirschhorn syndrome (WHS) is a genetic disorder due to partial deletion of the distal short arm of chromosome 4, with characteristic features including facial, cardiac and skeletal abnormalities, developmental delay, growth retardation, and a high mortality rate in the first two years of life. Though no known genes associated with immunodeficiency reside on the short arm of chromosome 4, these patients can present with immune dysregulation. Limited data shows patients with WHS most commonly have defects in humoral immunity, while T-cell immunity remains intact. To date, there has been one reported case of an isolated T-cell defect. We present the first reported case of a child with WHS with deficiencies in both humoral and cellular immunity. Methods: The patient is a 2 year old female with 4p16.3 chromosomal deletion and characteristic clinical findings consistent with WHS. She was referred to us for severe hypogammaglobulinemia noted on hospital admission for acute respiratory failure secondary to parainfluenza virus. Results: The patient has a history of methicillin sensitive S. aureus bacteremia, two episodes of pneumonia requiring intravenous antibiotics, recurrent acute otitis media infections requiring two sets of myringotomy tubes, and bacterial colonization of her bladder. Her childhood vaccinations are up to date. Immunologic evaluation revealed low IgG (185 mg/dL), IgM (13 mg/dL) and IgA (24 mg/dL), poor antibody response to diphtheria vaccination (0.02 IU/mL), and absent antibody response to tetanus (<0.1 IU/mL) and Prevnar 13 (0/23 serotypes protective) vaccines. Lymphocyte subset enumeration demonstrated decreased absolute CD3 (998 cells/mcL; normal 1400-3700), CD19 (154 cells/mcL; normal 390-1400) and CD8 (240 cells/mcL; normal 490-1300) counts. Mitogen stimulation, CH50, AH50 and MBL were normal. Random stool alpha 1antitrypsin level was normal. She began monthly immunoglobulin replacement and the severity of her infections has improved, with no further hospital admissions for bacterial illness. Conclusions: Immunodeficiency is a possible cause of early mortality in patients with WHS. This is the first report of a patient with WHS with deficiencies in both B-and T-cell lineages. Thorough immunologic evaluation and appropriate management for combined immunodeficiency may drastically improve survival and quality of life for affected patients. Bacitracin is often considered to be an innocuous over-the-counter medication. However, both T cell-mediated and IgE-mediated allergic reactions have been reported with increasing frequency, presumably due to its widespread use. We describe two cases of IgE-mediated reactions to bacitracin confirmed by positive skin prick testing (testing for second case in figure below). The first case is of a 53-year-old female who developed diffuse hives following bacitracin irrigation during breast augmentation surgery. The second case describes a 40-year-old man who developed anaphylaxis following the application of a topical bacitracin ointment to blistered skin. In both cases, the patient had a history of prior reaction to a triple antibiotic preparation before reacting to bacitracin alone. This case series demonstrates the variable routes of exposure causing allergic reactions, initially suspected neomycin allergy with resultant recommendation to use bacitracin alone, and the utility of skin prick testing to confirm bacitracin as the culprit allergen. Physicians need to be cognizant of the potentially serious consequences of using topical antibiotics on damaged skin and mucosal surfaces, and furthermore, consider bacitracin as equally important as neomycin in causing allergic reactions. Allergists can provide skin prick testing to the components of triple antibiotic ointments to confirm an IgEmediated mechanism for allergic reactions. Positive skin prick tests to bacitracin and bacitracin/polymixin Introduction: Bilateral periorbital swelling raises a wide differential diagnosis that includes both benign inflammatory conditions and malignant disorders. It may be a presentation of IgG4-related disease which is a recently emerged systemic condition characterized by unique lymphoplasmacytic infiltration of soft tissues by IgG4 plasma cells that can affect a wide variety of organs. Histopathologic and immunohistochemical analysis is important in the diagnosis of this disease and to differentiate it from other inflammatory and malignant conditions. We present a case of bilateral periorbital swelling which was initially diagnosed as organ limited vasculitis. However with further investigation he was diagnosed with IgG4-related disease. Case Report: Patient is a 21 year old male with no significant past medical history who presented with fluctuating bilateral eyelid swelling for 1 year. The swelling was associated with pain and redness isolated to around the eyes. Review of systems was negative for throat swelling, hives, fevers, chills, night sweats, or weight loss. He was initially placed on high dose prednisone which only temporarily improved his swelling. Shortly after the steroids were discontinued the swelling reappeared. MRI showed symmetric bilateral lacrimal gland enlargement. Labs including ESR, CRP, ANCA, ANA, and IgG4 level were within normal range. He had peripheral eosinophilia with absolute eosinophil count of 830. Initial lacrimal gland biopsy was read as non-necrotizing perivascular granulomatous infiltrate that involves small arteries and veins. He was initially diagnosed with Eosinophilic Granulomatosis with Polyangiitis. He had no other medical history or organ involvement suggestive of small vessel vasculitic syndromes. Biopsy slides were sent to a specialty laboratory who reported fibrosis, obliterative phlebitis, as well as 174 IgG4 plasma cells/high powered field consistent with the diagnosis of IgG4-Related Disease. He was subsequently treated with azathioprine and had significant improvement of his periorbital swelling. Conclusion: Integration of clinical, radiographic, and histopathologic data is necessary to diagnose IgG4-related disease. Biopsy with special staining for IgG4 plasma cells interpreted by an experienced pathologist is an important part of diagnosis and should be considered in any patient with bilateral lacrimal gland swelling to identify IgG4-related disease. Introduction: Common variable immunodeficiency (CVID) is the most common clinically significant primary immune defect. Although 70% to 80% of patients have had recurrent sinopulmonary infections, auto-immunity and inflammatory complications are also common. Case Report: We present the case of a 16 years old girl, who was admitted with a history of fatigue, weakness, pallor, edema of lower limbs. Physical examination was positive for Raynaud phenomenon. The diagnosis of systemic lupus erythematosus (SLE) was made based on 1. Proteinuria cellular casts, nephritis with low glomerular filtration rate, requiring replacement management with hemodialysis, 2. Hemolytic anemia and lymphopenia, 3. Pericardial effusion, 4. Central nervous system disorder with acute confusional state with cerebral MRI angiography showing irregular contours in distal segments and beading trend in vessels of medium and small size . Laboratory work-up showed Antinuclear antibody with a speckled pattern at a low titer; ANCA, anti DNA, lupus anticoagulant, B2GP1 were negative. Hypogammaglobulinemia and hypocomplementemia were found. Antibodies in the urine were positive for anti DNA at high titers, 333.2 U/ml (0-9.6U/ml): supporting the diagnosis of SLE.The patient had a torpid evolution, with severe sepsis, pancreatitis and gastrointestinal bleeding. She developed neurological impairment, seizures and Broca's aphasia, an MRI revealed ischemia in temporal and generalized vasospasm. Given the persistence of hypogammaglobulinemia at the expense of IgG and IgM as well as a history of multiple infectious events including pneumonia requiring mechanical ventilation, urinary tract infection by Pseudomonas aeruginosa and Serratia marscenses. Flow cytometry showing impaired lymphocyte subpopulation B (marked decrease in CD19 and CD20), lymphopenia at the expense of the subpopulation of T lymphocytes CD3 +, CD4 +. NK cells without alteration. Isohemagglutinins (anti A1) for blood groups reported in 1:16 dilution, and no response to immunization of Streptococcus pneumoniae. Conclusions: Clinicians should be aware about the relation between immunodeficiency and autoimmunity. Autoimmune diseases affect about 20% of CVID patients and are commonly the first manifestations of immune deficiency. The pathogenesis of autoimmunity in immune deficiency is unclear for the most part, further investigation must be done. Introduction: Bronchial thermoplasty (BT), approved by the US Food and Drug Administration in 2010, is a novel technique that uses radiofrequency energy during bronchoscopy to reduce the amount of built-up excess smooth muscle tissues and spasms in the airways of asthmatic patients. Several randomized clinical trials (AIR, RISA, AIR2 Trials) have reported the efficacy of BT on patients that have been unable to attain adequate control of their disease with high doses of inhaled corticosteroids and long-acting beta-agonists. The results from these studies demonstrated overall reduction of symptoms, reduction in the number of asthma attacks requiring emergency room care, and improvement in patients' AQLQ scores. Recently, the Global Initiative for Asthma has recognized BT as an add-on treatment option at the GINA Step 5 level for select adults with severe persistent asthma. We report a successful case of bronchial thermoplasty in a patient with severe-persistent atopic asthma. Case Report: We present a 47 year old Iranian American male with allergic rhinitis, nasal polyposis, obstructive sleep apnea, reflux disease, and severe persistent allergic asthma who underwent bronchial thermoplasty. Previously, his asthma remained difficult to control despite multi-drug therapy [montelukast,mometasone furoate/ formoterol fumarate dihydrate, theophylline, tiotropium bromide, loratadine, olopatadine nasal spray, fluticasone furoate nasal spray, and omalizumab]. Prior to the procedure, he had an average FEV1 of 40%, ACT scores <19, and levalbuterol use 3-6 times a day for relief. Since the completion of the BT procedure 1 year ago, the patient has been able to decrease his levalbuterol use to about once a day, improve his latest FEV1 to 70%, and ACT score to 25. He has not experienced severe exacerbations requiring ER visits or hospitalization over the past 12 months. Furthermore, he has been able to discontinue several of his previous medications, such as montelukast, tiotropium, and theophylline. Conclusion: This is the first case report of an atopic asthmatic patient successfully treated by bronchial thermoplasty after failure of conventional and several non-conventional therapies. This case demonstrates that for some highly-select adult patients with difficult to manage allergic asthma, a referral to an asthma specialty center with bronchial thermoplasty should be considered Objective: To assess the safety of replacing angiotensin-converting enzyme inhibitors (ACEI) with angiotensin II receptor blockers (ARBs) in patients with a history of ACEI induced angioedema. Angioedema has been associated with ACEI use in hypertension treatment. Angiotensin II receptor blockers have long been considered a safe alternative treatment for hypertension in cases of ACEI induced angioedema. Angiotensin II receptor blockers selectively block the binding of angiotensin II to the AT1 receptor and therefore do not cause accumulation of kinins which subsequently lead to angioedema. Methods: Case Report Results: A 57 yr old Caucasian male experienced an acute episode of angioedema with laryngeal edema. He experienced swelling of his tongue, lips and larynx. The angioedema failed to respond to two doses of epinephrine and IM diphenhydramine. He required a tracheostomy and ICU admission for 14 days. The patient was discontinued from his home medications, which included irbesartan (Avapro). The angioedema persisted for 7 days, and failed to respond to I.V. corticosteroids and antihistamines. His history revealed acute episode of angioedema 5 years prior during a surgical procedure. The patient was under conscious sedation, and he experienced unilateral tongue swelling upon awakening. He was being treated with lisinopril at the time for hypertension and was switched to irbesartan. Subsequently, the patient claimed he experienced intermittent episodes of lip swelling. He was evaluated for Hereditary Angioedema, and all labs were within normal limits. Immunocap to food and environmental allergy was unremarkable as well. An autoimmune panel was also negative. He has not experienced any episodes of angioedema since being discontinued from irbesartan. Conclusions: The patient's angioedema appeared to be related to his ARB therapy. In the ONTARGET trial, the incidence of ARB induced angioedema was 0.1 % compared to 0.3% for ACEI associated angioedema. Although the incidence of ARB induced angioedema is lower than ACEI, life-threatening complications such as laryngeal edema may result. The mechanism of ARB associated angioedema has not been clearly established, but studies have shown that ARBs may also increase bradykinin levels. Therefore, other classes of anti-hypertensive medications should be considered as first line replacements in patients with a history of ACEI induced angioedema. BLACK RUBBER AND P-PHENYLENEDIAMINE CONTACT DERMATITIS. C. Lin * , M. Frieri, East Meadow, NY. Introduction: Contact dermatitis is a common skin inflammation that occurs when foreign substances irritate the skin, characterized by erythematous and pruritic lesions. Contact dermatitis has been well documented in over 3000 chemical triggers. P-Phenylenediamine, as a chemical substance is widely used in hair dye. Its partially oxidized state can cause allergic reactions in sensitive individuals. Case: A 61 year old white female was referred to the allergy clinic from the emergency department for an intensely diffuse rash on her skin disseminated from the neck to waist for 2 weeks. She also complained of a pin and needle sticking pain within the rash area. She had hair dye at a salon 2 days before she developed the pain and skin reaction. There was no shortness of breath, stridor or throat symptoms. Past medical history included seasonal allergy and food allergy. Skin prick tests revealed positive for grass, trees and dust mite. She takes cetirizine all year around for allergy prophylaxis. On physical examination, she was alert, oriented, and afebrile with stable vital signs. Her skin showed an extensively maculopapular rash randomized throughout from her neck to chest and back area. Methods: A skin patch test disclosed a positive test for p-Phenylenediamine and black rubber mix. sIgE testing for latex was negative. She was advised to avoid known allergic related chemicals, continue to take cetirizine or fexofenadine. The rash and pain gradually improved. Conclusion: Contact dermatitis can result from hair dye hypersensitivity. Its severity ranges from a mild localized rash to a widespread allergic dermatitis reaction or even rarely, anaphylaxis. The diagnosis relies upon a detailed history and physical examination. The skin patch test remains the gold standard to confirm contact allergic reactions. Avoidance of exposure to rubber products, causative hair dye should be withdrawn. Systemic anti-histamines are often needed and in severe patients, a short course of systemic steroids may be required. Prevention could be achieved by testing oxidative dyes before use or switched to non-oxidative hair dye. Patients should also be careful with rubber products and cross-reacting chemicals or drugs such as printing inks, temporary black henna tattoos Introduction: Drug allergy can limit essential therapeutic options for patients. Hypersensitivity to monoclonal antibodies has been documented in adult patients, but desensitization to this class of drugs has not been well described in the pediatric population. Here, we present a pediatric patient with hypersensitivity to rituximab who underwent successful drug desensitization. Methods: Desensitization to rituximab was completed via a modified 12-step, 3-bag method in an inpatient unit. The protocol started at an initial dose of .0057 mg with doubling of the dose at 20-minute intervals, with the proposed final step to be infused at 75 cc/hr to provide at total therapeutic dose of 285 mg. The child developed significant rash prior to the last step, and thus future protocols reduced the infusion rate of the last step. The patient was monitored after completion of each desensitization protocol. Results: A 7-year-old boy with neonatal biliary atresia status post remote liver transplant developed EBV negative post transplant lymphoproliferative disease (PTLD). Rituximab were required for treatment of the child's PTLD, but he developed urticaria and cough with infusions despite premedication with antihistamines. As rituximab was a critical part of the child's chemotherapy, urgent desensitization was required. The patient underwent the desensitization protocol, but developed diffuse skin erythema prior to completion of the last dose. The protocol was therefore modified as described above, with increased premedication. The child tolerated subsequent desensitization protocols, and completed his chemotherapeutic regimen. Conclusions: This is one of the only reported cases of successful desensitization to rituximab monoclonal antibody in a pediatric patient, and includes strategies for patients who have reactions during the procedure. This protocol will be beneficial for future patients with rituximab hypersensitivity. Introduction: Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) is a primary immune deficiency (PID) associated with autoimmune sequelae; however, chronic idiopathic urticaria (CIU) is a very rare manifestation. While omalizumab is effective in patients with CIU, its mechanism of action in these patients is not completely understood and its role in autoimmunity and anti-FceRI autoantibodies is unclear. We present a case of CIU in an APECED patient refractory to standard therapy that resolved with omalizumab. Case: APECED was diagnosed at age 9 after an ICU admission for severe hypocalcemia and tetany secondary to hypoparathyroidism. Genetic testing revealed the patient was a compound heterozygote with muta-tions R257X and c.967_979del13 in the AIRE gene. The patient's clinical manifestations included chronic mucocutaneous candidiasis, hypoparathyroidism, adrenal insufficiency with positive 21-hydroxalase antibodies, vitiligo, hypoplastic dental and nail enamel, and delayed growth. At age 13 she developed chronic urticaria despite high dose antihistamines and daily montelukast. Laboratory workup revealed the presence of anti-FceRI autoantibodies without progression of autoimmune disease. Treatment with omalizumab 150mg led to resolution of symptoms after one month and reduction of daily oral medications. The patient is currently under treatment every 8 weeks with omalizumab and will stop therapy soon to evaluate for possible remission of her urticaria. Conclusions: Omalizumab appears to be an effective therapy for CIU in APECED; however, questions remain regarding its underlying mechanism of action. The rare appearance of CIU in this patient population seems to run counter to the hypothesis that CIU is associated with autoimmune disease. Our patient highlights the need for further research to better characterize the relationship of CIU with immune dysregulation and how therapy with omalizumab influences this process. Background: Systemic mastocytosis (SM) can be associated with clonal hematological disorders like myelodysplastic syndrome (MDS) and acute myeloid leukemia (AML). Most of the SM-AML cases have translocation t(8;21) and mutation of c-kit gene. Rarely, specific SM treatment is required. We present a patient with SM-AML without t (8; 21) and c-kit mutation who developed refractory hypotension due to mast cells mediators release. Methods: case report; review of literature Case Report: 53 year old female was diagnosed with myeloproliferative-myelodysplastic syndrome which evolved to high risk MDS with complex cytogenetics (deletion of chromosomes 5,7 and trisomy 8) for which she received induction chemotherapy. After discharge she developed urticaria that slowly responded to daily antihistamines and was thought to be medication related. She relapsed with AML (similar cytogenetics) in 3 months and received re-induction therapy. The course was complicated by Enterobacter bacteremia that cleared with daptomycin. However, she developed severe persistent hypotension along with new diffuse pruritic maculo-erythematous rash. Skin biopsy showed spongiosis. The patient was transferred to intensive care unit requiring vasopressors which were difficult to taper off. Cardiac and septic shock were excluded as causes for refractory hypotension. Repeat BM biopsy for induction response revealed clearance of myeloid blasts but showed 20% CD 117 + oval spindle-shaped mast cells, which were positive for tryptase and negative c-kit mutation. Serum tryptase level was 42 ng/ml. The patient was diagnosed with SM-AML and started on histamine-1, histamine-2 and leukotriene receptors blockers with significant improvement in blood pressure and skin rash. After 2 weeks she died from multiorgan failure. Conclusion: This is the first reported case of refractory hypotension due to mast cells mediators release in a patient with SM-AML. Moreover, no cases of SM-AML without t(8;21) or c-kit mutation were previously reported. From the sequence of clinical events we believe that the mast cells were uncovered by the treatment for AML. As a result, the diagnosis of SM was made after cytoreductive therapy. It is imperative that clinicians are aware of coexistence of SM in clonal malignant hematological disorders. If clinically suspected, prompt treatment improves the SM-related symptoms. Color additives are frequently used in processed foods to impart color and enhance its appearance. The US Food and Drug Administration (FDA) is responsible for regulating color additives in commercial foods to ensure safety and accurate labeling of approved ingredients. Synthetically produced colors are FDA-certified and listed in food labels. However, natural color additives are exempt from FDA-certification and labeled as natural color. Annatto is a natural color additive extracted from the seeds of Bixa Orella trees that imparts an orange-yellow color to foods. The prevalence of adverse reactions to food additives in the US is less than 1% in adults but 2-7% in children and higher in those with atopy. We present an IgE mediated reaction to annatto in a 4 y/o atopic child. She developed urticaria within minutes of eating mac and cheese at a chain restaurant on two separate occasions. She also had urticaria with angioedema immediately after eating ice cream sandwiches, pasteurized cheese and boxed cheeseburger pasta. She tolerates chocolate, peanut, hazelnut, milk, wheat and egg ingredients in above foods without reaction. Ingredient review for the mac and cheese and ice cream sandwiches both contained annatto seed. The boxed cheeseburger pasta only listed natural flavors and coloring. Skin prick test to annatto extract was positive (8x30 mm) compared to negative control (5x9 mm). Use of annatto as natural alternative to artificial color is exempt from FDA certification and gaining popularity. It is labeled as all natural, no artificial food coloring, achiote, pimentao, bixin or norbixin. Annatto is used in cheeses, dairy spreads, rice, custard powder, baked goods, snacks, cereals and smoked fish. Despite its widespread use, there have only been 2 cases in adults of IgE mediated reaction to annatto. We present the first case of IgE mediated reaction to annatto in a child. Our patient had no other food allergies and the timing of ingestion of annatto-containing food and development of symptoms with a positive immediate hypersensitivity skin test to annatto extract is highly suggestive of an IgE mediated reaction. It is increasingly important to investigate reactions to natural food coloring in patients with symptoms to multiple unrelated foods. As advocates for our patients, allergists should advocate for FDA certification and specific labeling for all natural flavors and coloring. Background: Dermatomyositis (DM) is an idiopathic autoimmune myopathy that predominantly targets the skin and skeletal musculatures. This report describes an unusual case of a patient having both Extraovarian Primary Peritoneal Carcinoma (EOPPC) and Abdominal Cocoon Syndrome (ACS) who initially presented with Dermatomyositis. Case Summary: This is a case report of 47-year old woman admitted due to rashes. History revealed that 7 months prior to admission, there was note of multiple, pruritic erythematous macula rashes prominent on the face, neck and chest. She was admitted and managed as case of hypersensitivity reaction. Six months prior to admission, the skin lesions over the face and chest recurred associated with raised violaceous, scaly papules on the metacarpophalangeal, proximal interphalangeal and distal interphalangeal joints on both hands and erythematous, cracked and roughened periungal areas. Patient was readmitted and diagnosed with dermatomyositis. She was started on prednisone and methotrexate. Three months prior to admission, an abdominal distention associated with difficulty of breathing was noted and underwent abdominal CT scan revealing moderate ascites thus paracentesis was done. Ascitic fluid analysis was positive for malignant cells. Medical oncologic consult was sought. Patient came in with BMI of 18.3 kg/m2, pale conjunctivae, macular hyperpigmented lesions at the neck and shoulder area, scaly papules at the MCP, PIP, DIP of both hands, distended abdomen with shifting dullness and grade 1 pitting bipedal edema. CA-125 was elevated among the requested tumor markers. Exploratory laparotomy with tenckhoff catheter insertion was done with. intraoperative findings of encapsulated mass, thick walled containing the bowels adherent to the anterior abdominal wall and sidewalls and foul smelling ascitic fluid. Uterus and both ovaries were atrophic. Biopsy of peritoneal tissue revealed psammoma bodies. With these pertinent findings, EOPPC and ACS were considered. Patient endured cycles of chemotherapy using Carboplatin and Paclitaxel. Conclusion: The incidence of classic DM is 5.5 cases per million per year. The reported frequency of malignancy varied from 6% to 60% with most large population based cohort studies revealing a frequency of about 20 to 25%. Objective: To assess the appropriate evaluation and management options for a patient with Seabather's Eruption (Sea Lice). Seabather's Eruption is an entity that should be included in the differential diagnosis of patients presenting with rashes and appropriate supportive care should be offered. Methods: Case Report Results: This is a 12 year old female from South Florida that presented with a rash that started under her bathing suit top after going swimming in the ocean. The rash was maculopapular and pruritic. It spread from her torso to her hands and feet and then became generalized with sparing of the head and neck. Symptoms started about 2 hours after coming out of the ocean. Patient had gone into the ocean previously even swimming at the same general beach area without incident. Patient also claimed that her family was with her in the same ocean water and did not develop the same symptoms. Crusting of the rash with associated blistering with additional pruritis and discomfort followed over the next 3-4 days. Vital signs were stable. There was blistering and crusting noted on the skin on physical exam. Patient was given hydrocortisone 2.5% ointment to be applied topically in a thin layer to the affected areas as needed only and placed on cetirizine 10 mg po daily for 2 weeks duration. Discussion: Seabather's eruption is a self limited reaction that results in a highly pruritic papular rash on exposure to seawater. It results from a hypersensitivity to thimble jellyfish larva (Linuche Unguiculata). Cases are noted from March to August but peak incidence is in May and June. The eruption is seen in the United States east coast of Florida. Bathing suits are believed to trap the larvae which then discharge a venom, the significance of which is unknown. Swimmers washing themselves off with fresh water with their bathing suits off and those that refrain from wearing their bathing suits for an extended period of time are found to be better protected against Seabather's Eruption. Conclusion: Providers should be careful to avoid narrow differential diagnoses when patients present with rashes. Seabather's eruption should be considered in the differential in patients presenting with pruritus and papular rash and proper history and physical exam should be conducted to ascertain relevant information for this diagnosis. Supportive measures with symptomatic care is the treatment of choice. Rationale: Drug allergy consults are challenging due to the dearth of published data. Furthermore, there are few international journals who publish case reports of drug allergies. This study aims to determine the utility of a website-based database of drug allergy case reports and desensitization protocols among American allergists. An online survey was administered to American allergists, who are members of the American Academy of Allergy, Asthma and Immunology (AAAAI). Methods: A semi-structured survey was created and administered to members of the AAAAI. The questions were split into two parts: demographics and database design. The demographics section of the survey strives to ascertain the types of drugs commonly consulted for and the frequency of these consults. The next section attempts to elucidate how American allergists proceed with desensitization protocols-earlier research with Canadian allergists showed that personal experience was the most used resource. Results: 193 members of the AAAAI answered the survey. In terms of what percentage of consults were related to drug allergies 62.1% estimated 1-10%, with 22.6% estimating 10-25%. When discussing which drugs patients are consulted about-the vast majority (>95%) said they had been consulted for Peni-cillins, and Cephalosporins. Similarly when asked which drugs they had been consulted for a desensitization protocol 93.1% of respondents named penicillin. When asked what resources they use for researching desensitization protocols 82.8% said specific E-Journals, 76.6% said E Journal search database, and 69.3% use online search databases such as google or Uptodate. When asked if they would utilize a website database of case reports on drug hypersensitivity reactions and desensitization protocols 82.9% responded yes with 54.1% of respondents also being interested in submitting to the database. In discussing the characteristics that would be most important in the database respondents felt overwhelmingly (>85%) that it should be structured and contain information pertaining to patient demographics, medication usage, desensitization protocols and patient presentation. Conclusion: There is widespread support among American Allergists for the creation of a database of drug allergy. The next logical step is to gather support to create this database, which will require a multistakeholder approach. Introduction: The pungency of extra virgin olive oil (EVOO) is attributed to one of its phenolic compounds called oleocanthal (OC) and is mediated through the transient receptor channel potential cation channel ankyrin subtype 1 (TRPA1) expressed in the posterior oropharynx. EVOO also mimics the pharmacology of ibuprofen in that both agents cause a dose-dependent inhibition of cyclooxygenase enzymes COX-1 and COX-2. EVOO sensitivity has been described in the literature previously however EVOO desensitization has not. Herein we report the first case of successful EVOO desensitization using a novel EVOO desensitization protocol. Case Description: A 25-year-old patient with medical history significant for aspirin sensitivity, exercise induce asthma and mixed vasomotor and allergic rhinitis presented with a history of facial and tongue pruritus, throat irritation, coughing and hives within minutes of olive oil consumption. Symptoms required diphenhydramine however several prior episodes required epinephrine. A series of food and drug challenges were entertained to establish the mechanism of EVOO sensitivity in this patient. Also a novel EVOO desensitization protocol was devised. Discussion: EVOO desensitization has not been attempted previously in the literature and in this report we describe the first case of successful EVOO desensitization using a novel EVOO desensitization protocol. Furthermore the patient continues to be in a desensitized state 2 years after the procedure maintained with daily doses of 10 ml EVOO. Although the mechanism of her reaction is likely due to OC stimulation of TRPA1, the mechanism of desensitization and maintenance of the desensitized state is unclear and requires further study. A.S. Chau *1 , L. Helfner 2 , V.R. Bonagura 2 , A.M. Jongco 2 , 1. Mineola, NY; 2. Great Neck, NY. Background: Mast cell activation disorders (MCAD) affect multiple systems and are caused by episodic mast cell mediator release. Intrinsic defects in mast cell proliferation or activation are found in primary MCAD such as systemic mastocytosis (SM) and monoclonal mast cell activation syndrome. In secondary MCAD such as allergic disorders, mast cells that are normal in quantity and function respond to external stimuli (i.e., allergens). In idiopathic MCAD, such as mast cell activation syndrome (MCAS), there is no identifiable cause of mast cell activation. We describe the longitudinal clinical course and management of a patient with MCAS. Methods: A 49-year-old woman diagnosed with autoimmune disorder, not otherwise specified, status post thymectomy originally presented with chronically elevated IgE and ANA, alopecia universalis, weight loss, chronic idiopathic urticaria and multiple food sensitivities. Extensive evaluation revealed multiple aeroallergen hypersensitivity and unremarkable bone marrow studies. There was no evidence of immune deficiency, autoimmunity, IgE-mediated food allergies, SM and celiac disease. Results: Urine histamine and histamine release assay were normal. Serum tryptase was unremarkable repeatedly. Her symptoms did not improve after trials of immunosuppressive and immunoglobulin replacement therapy. In collaboration with the Center of Excellence for Mastocytosis and Mast Cell Activation Disorders at Brigham and Women's Hospital, she was started on an aggressive anti-inflammatory regimen including H1 and H2 antihistamines, cromolyn, monteleukast and omalizumab more than three years ago. This resulted in sustained weight gain, improved tolerance to previously sensitive foods and aeroallergens, minimal hair regrowth, and decreased frequency and severity of urticaria. Despite improvements on optimal medical therapy, overall symptom control and quality of life remain poor. The patient is considering other treatment modalities including allogeneic hematopoietic stem cell transplantation, which has been described in two small series for SM with variable outcomes. Conclusions: MCAS is a diagnosis of exclusion that warrants consideration in patients with chronic multisystem complaints of unclear etiology. MCAS diagnosis and management are evolving; additional research is necessary for understanding the etiology and natural history of this disease. Introduction: Breastfeeding anaphylaxis is rare, but potentially fatal. Symptoms range from urticaria to hypotension and syncope and occur in close relation to lactation. The pathogenesis of the reaction remains unclear; however, hormone shifts related to the end of gestation and the beginning of lactation seemingly play a role. Waning levels of progesterone postpartum and loss of its mast cell stabilizing effect has been one hypothesized mechanism, though the roles of oxytocin, prolactin, adrenocorticotrophic hormone (ACTH), corticotropin-releasing hormone (CRH) and increased numbers of antepartum mammary and uterine mast cells have also been questioned. In addition, NSAIDs may also be a contributing factor. Methods: Presentation, evaluation, diagnosis and management of a patient with breastfeeding anaphylaxis. Data: A 25 year old gravida 3 para 3 woman was admitted with anaphylaxis on postpartum day 3 following the birth of her third child. She had breastfed her first child for 2 months without difficulty. Three days after the delivery of her second child, she developed isolated urticaria after breastfeeding. She discontinued breastfeeding and symptoms resolved. Her third child was born via uncomplicated vaginal delivery. She had been taking 600 mg of ibuprofen every 8 hours following the delivery, but was on no other medications. Breastfeeding was uneventful for the first 48 hours after delivery. On postpartum day 3, however, with the first breastfeeding after generation of mature milk, she developed diffuse urticaria, tongue swelling, emesis, and dizziness. On admission, she was tachycardic, tachypneic, and hypotensive. A tryptase level obtained 6 hours after the episode was elevated at 14.6 ng/mL (normal < 11.5 ng/mL). The patient desired to continue breastfeeding. She was placed on prophylactic cetirizine 10 mg BID and all NSAIDs were discontinued. She subsequently breastfed successfully for 24 hours while under close observation. Conclusions: Our patient had anaphylaxis with breastfeeding on postpartum day 3 when lactogenesis was established. She subsequently tolerated 24 hours of breastfeeding without difficulty with the use of cetirizine 10 mg BID and avoidance of NSAIDs. Our case suggests that mothers with a history of breastfeeding anaphylaxis and a desire to breastfeed can likely do so with appropriate treatment and counseling. Objective: To determine the safety of replacing mesalamine with other 5aminosalicylic (5-ASA) formulations in patients with hypersensitivity to mesalamine. Patients with inflammatory bowel disease (IBD) such as ulcerative colitis are usually treated with 5-ASA preparations such as mesalamine. These drugs are first line medications used to treat mild to moderate ulcerative colitis due to their steroid-sparing effects. Balsalazide is a dimerized 5-ASA preparation that may be tolerated in patients with hypersensitivity to mesalamine. Due to its structural differences, dimerized 5-ASA medications may be considered to be safe alternatives due to a presumed lack of crossreactivity. Methods: Case Report Results: A 39-year-old Caucasian female presented to our service after experiencing anaphylaxis following use of mesalamine (Asacol). She had been using mesalamine for 19 years to control her mild to moderate ulcerative colitis. Six months ago, she experienced diffuse urticaria and generalized pruritus one hour after taking her morning dose of mesalamine. She took diphenhydramine, and her symptoms resolved within hours. The next day, she took her morning dose of mesalamine and experienced immediate anaphylaxis. She developed laryngeal edema, diffuse urticaria, and generalized pruritus. The patient was treated with epinephrine, and her symp-toms resolved. The patient underwent a provocative drug challenge with balsalazide (Giazo) in our clinic over two days. The patient reacted on the second day when she experienced swelling of her hands and generalized pruritus. The patient was administered oral antihistamines and corticosteroids and her symptoms resolved within four hours. Conclusions: Cross-reactivity may occur between different 5-ASA preparations despite their structural differences. Patients with inflammatory bowel disease who are hypersensitive to mesalamine may also react to balsalazide and olsalazine. Mesalamine hypersensitive patients should not be started on these medications without undergoing a provocative drug challenge. We designed an outpatient protocol that will safely enable patients to undergo testing in an office setting. Introduction: Allergists are commonly asked to evaluate the etiology of tongue swelling. We report a case of a woman with 16 days of tongue swelling secondary to endotracheal tube (ETT)-induced vascular obstruction of the base of the tongue. Methods: Case Report. Data: A 55 year-old female with systemic lupus erythematosis, end-stage renal disease, hypertension, and congestive heart failure presented to the emergency department with acute onset shortness of breath and somnolence. MRI revealed bilateral intraventricular hemorrhage and chest imaging revealed pneumonia. She was intubated secondary to respiratory distress. Tongue swelling was reported 2 days after intubation and worsened over the next 2 days. Physical examination revealed a swollen tongue, which was at least 2-3 times the size of normal, protruding out of her mouth. Pressure ulcers were noted on the base of the tongue. Laboratory evaluation revealed normal C3 and C4. Due to concern for oropharyngeal edema, a tracheostomy was performed. Swelling had not improved 14 days after intubation and a 4 day course of dexamethasone was administered without improvement in swelling. Tongue swelling improved 1 day post-extubation and resolved within 2 days. Past medical history was significant for lisinopril-induced angioedema. The patient did not receive an ACE-inhibitor or ARB prior to or during admission and medication regimen did not change prior to or post-resolution of swelling. The differential diagnosis of tongue swelling includes hereditary angioedema (AE), acquired AE, medication-related AE, mechanical obstruction, and sublingual hematoma secondary to a difficult intubation. Tongue swelling was attributed to venous obstruction secondary to local mechanical compression of the base of the tongue by ETT. This is supported by a normal C4, onset of swelling after ETT placement and resolution after ETT removal, and the prolonged course of swelling (16 days). Introduction: Immunoglobulins (Ig) are routinely used in management of Primary Immune Deficiency diseases (PIDD). Ig formulations differ based on the content of IgG, IgA, osmolality and stabilizer. Most adverse reactions to IVIG are either due to a rapid rate of infusion or IgA deficiency. We report a case of hypersensitivity reaction to the stabilizer in the Ig formulation. Methods: A 50 year old female with a 3-year history of CVID was referred by her allergist for management of hypersensitivity reaction to subcutaneous immunoglobulin. She had been treated with subcutaneous hizentra weekly for 2 years; however, 2 months ago she developed an urticarial rash 20 minutes after taking her usual dose. Subsequent doses caused immediate symptoms of throat closure, difficulty breathing, swallowing and low blood pressure requiring treatment with epinephrine, prednisone and antihistamine in the ER. She denied symptoms of nausea, vomiting, fever, chills or headache. Hizentra was subsequently discontinued and she was referred to UTMB for further evaluation. On evaluation, her history was negative for allergic or anaphylactic reactions to other medications and IgA deficiency was ruled out. Skin prick testing to Ig was not helpful due to dermatographism. The patient was treated with Gammagard, chosen for the differences in osmolality and stabilizer content vs. Hizentra. Patient received a slow infusion of Gammagard and was subsequently switched to SC administration without further reactions. Discussion: Hizentra contains the stabilizers L-proline and polysorbate 80, whereas Gammagard contains only glycine. Polysorbate 80 is commonly used in vaccines, chemotherapy and tablet formulations. It has been reported to cause non IgE mediated anaphylactic reactions in patients receiving Docetaxal and is the most likely cause of hypersensitivity reaction in our patient. No hypersensitivity reactions with L-proline have been reported in literature. Hizentra also has higher osmolality as compared to Gammagard but the adverse reactions due to high osmolality usually include thrombosis and kidney injury which were not present in our patient. Conclusion: We report a rare case of hypersensitivity reaction to a stabilizer in Hizentra, an Ig formulation used for treatment of PIDD. This report alerts the allergist/immunologist about work up and management of patient's with hypersensitivity reaction to an immunoglobulin formulation. L. Finkas * , R. Katial, Denver, CO. Introduction: A rare case of eosinophilic cholecystitis in a patient with AERD Case: 50-year-old male with past medical history of asthma, chronic sinusitis with nasal polyps, and AERD. He underwent aspirin desensitization a few years prior and had improvement in both his asthma and sinus disease. Following desensitization, he was maintained on aspirin 650 mg BID and was subsequently decreased to 325 mg BID. He presented for routine 6 month follow up at which time he had been experiencing significant gastrointestinal symptoms for the previous 3 weeks. His symptoms began as indigestion and heartburn and progressed to diarrhea and vomiting. His PMD had placed him on a course of antibiotics, but despite antibiotics, he continued to have symptoms. Stool studies were obtained and C-diff was negative. His aspirin dose was decreased to 81 mg without improvement in GI symptoms. Further diagnostics were pursued and complete blood count was notable for an elevated WBC of 14,000 with an absolute eosinophil count of 6100. Liver function tests, ESR and CRP were within normal limits. He was evaluated in the emergency room given his persistent symptoms and was found to have cholecystitis. He underwent a cholecystectomy and pathology revealed transmural inflammatory cell infiltrate composed predominantly of eosinophils. Bone marrow biopsy, EGD and colonoscopy were performed for possible hypereosinophilic syndrome. His bone marrow biopsy was negative for FIP1L1/PDGFRA mutation and was only notable for mild eosinophilia. EGD and colonoscopy were negative for eosinophilic infiltrate. He remained on aspirin during this time and following his surgery increased to 325 mg daily. Following cholecystectomy, the patient had significant improvement in his peripheral eosinophilia and his eosinophil count retuned to his baseline of 400. His gastrointestinal symptoms resolved. Conclusion: This is the first case of a rare eosinophilic process isolated to the gallbladder in a patient with AERD, which in itself is manifested by increased circulating eosinophils in over half the patients. The authors have previously reported rare complication of pancreatitis after aspirin desensitization and a few unpublished cases of increased GI symptoms with eosinophilia. This shows another possible complication in AERD patients with underlying eosinophilia. C. Dutmer * , E. Gelfand, Denver, CO. Introduction: Cystic fibrosis (CF) is a disease caused by mutations in the CF transmembrane conductance regulator (CFTR) gene, resulting in deleterious effects on epithelia of various tissues. Though the classic CF phenotype is characterized by progressive pulmonary disease and associated exocrine pancreatic insufficiency in childhood, disease phenotype varies greatly by specific CFTR mutations, associated genetic modifiers, and environmental factors. Nearly 2,000 CFTR gene mutations have been identified, highlighting the allelic heterogeneity in the CFTR gene. Discovery of new mutations (and associated factors) may explain the diverse nature of CF-related disease phenotypes with varying age of onset and recognition. We describe a 59-year-old woman evaluated for immune deficiency in whom CF was diagnosed. Case Description: A 59-year-old Caucasian woman with chronic obstructive pulmonary disease and a 40 pack-year cigarette smoking history presented with an 18-month history of recurrent pneumonia and sinus disease as well as an episode of pancreatitis. Prior history was notable for the absence of significant infection. She recently required numerous hospitalizations and had subsequently required supplemental oxygen while suffering from chronic dyspnea and cough. Expectorated sputum and bronchoalveolar lavage bacterial cultures revealed mucoid Pseudomonas aeruginosa. Cylindrical and varicoid bronchiectasis as well as chronic maxillary sinusitis were evident on CT (Image 1). Quantitative serum immunoglobulin levels were normal as was the remainder of her immune evaluation. Family history identified a paternal first cousin once removed with CF as well as carrier status for a CF gene mutation in the patient's daughter. Sweat chloride testing was elevated at 72 mmol/L and CFTR gene sequencing analysis revealed two mutations: deltaF508 and R117H. Discussion: New-onset sinopulmonary infections in the 6 th decade of life of a long-standing smoker prompted initial concern for secondary or acquired immune deficiency. Con-sideration of CF as an explanation of recurrent sinopulmonary disease in a previously "healthy" adult is certainly unusual. Growing evidence of the diverse genotype-phenotype relationships in CF and reported variability in diseaseonset suggests that a diagnosis of CF be evaluated in adults with recent-onset pneumonia, bronchiectasis, and sinopulmonary disease. Introduction: According to package inserts, use of Diprivan brand propofol or Fresenius brand propofol is contraindicated in patients with soy or egg allergy. This is due to the use of soybean oil and egg lecithin as excipients and the potential for soy or egg protein contamination. Fresenius is additionally contraindicated in peanut allergic patients due to potential cross-reactivity between peanut and soy. Case Report: A 9-year-old girl with history of Chiari malformation was noted to have swelling of her face, lips, and tongue after neurosurgery. She had a history of identical reactions to fresh egg and peanut, and anesthesiology suspected her reaction was due to Fresenius and her peanut allergy. She had tolerated Diprivan at least 7 times before, most recently 2 months prior. She was given H1 and H2-blockers and was extubated postoperatively, but then developed stridor, wheezing, desaturations, and again had lip and tongue swelling. She continued to be symptomatic despite anaphylaxis management and required an epinephrine drip and nasal intubation. She required a slow 7 day wean off epinephrine. Results: Total tryptase level drawn at hour four was 4 ng/mL. C4 and C1 esterase inhibitor levels were normal. IgE levels for egg white, egg yolk, and soy were negative, while peanut was positive at 1.27 kU/L. Follow up skin testing to egg and soy were negative, and peanut was positive (5 mm wheal). Discussion: After literature review, we found that the vast majority of patients with soy, egg, or peanut allergy receive propofol without issue. In one study, there were no adverse reactions to propofol amongst 45 food allergic patients. Despite its contraindication in peanut allergic patients, Fresenius does not contain any ingredients which make it more allergenic than Diprivan in patients with egg, soy, or peanut allergy. While we have not yet been able to identify the cause of our patient's protracted reaction, we do not suspect it was related to her peanut allergy. Conclusion: Care should be taken to understand the reasoning behind food allergy contraindications of medications, as these can often be given to food allergic patients without issue. Such contraindications may cause unnecessary avoidance of medications and/or may distract from the identification of a true medication allergy. Introduction: In a country where the transmission of many diseases has been largely eliminated by vaccination, the widespread outbreak of preventable illness could modify management and treatment options of patients with hypogammaglobulinemia. We describe two patients with a history of hypogammaglobulinemia who were in the process of transitioning off IgG replacement. In light of the measles and mumps outbreak in Ohio, these patients were either continued or restarted on IgG replacement. This was due to their unvaccinated status and the recommended interval between receiving antibody-containing products and administration of a measles-containing vaccine. Case Reports: Case 1 involves a 5 year-old female who was introduced to the Immunology service at 5 weeks of age during an admission for bronchiolitis. Immunology was consulted for recurrent respiratory infections. Her clinical course was complicated by pneumothorax, bronchopleural fistula, and necrotizing pneumonia. Her immune workup was normal except for a gradually declining IgG level. Stool α-1-antitrypsin initially high, favoring the diagnosis of hypogammaglobulinemia secondary to a protein-losing enteropathy. She started IgG replacement at 15 months. By 4 years of age, there was discussion to begin weaning IgG replacement. This was halted due to concern about the measles and mumps outbreak. Case 2 is that of a 2 year-old male who presented in the first month of life with enterocolitis and dehydration. He had a low total protein, albumin, and IgG level thought to be due to GI losses, although stool α-1-antitrypsin negative. Work-up for a unifying diagnosis was negative. History now thought to be most consistent with transient hypogammaglobulinemia of infancy. He was started on IgG replacement at 4 months. At 2 years he was weaned off IgG replacement and remained off for 6 weeks. Secondary to concern over the increased risk of exposure to measles or mumps through his particular daycare setting, IgG replacement was restarted. Conclusions: These cases highlight the challenges that exist for our patient population with Immunoglobulin Deficiency due to the reemergence of vaccine-preventable illness. As these exposures continue to increase, we must modify our treatment plans to provide optimal care and ensure patient safety. Introduction: Food is the most common cause of anaphylaxis (Lieberman P et al. J Allergy Clin Immunol. 2010). Very few cases of mustard seed induced anaphylaxis have been reported. We present a case of mustard seed anaphylaxis. Methods: A case report Results: 29 year old man presented to our department for evaluation of anaphylaxis. He had an episode of generalized urticaria ( Figure A) , chest tightness, vomiting, facial flushing and angioedema ( Figure B) , within 30 minutes of eating a prepared quinoa dish at home. He did not seek medical attention and his symptoms resolved within hours of self-administration of loratadine and diphenhydramine. He had no relevant past medical history of previous adverse reactions to foods. Upon further questioning, the ingredients in the quinoa dish included: chicken, rice, avocado, ginger, onion, potatoes, tomato, nutmeg, yeast, soy, wheat, black pepper, thyme, and mustard seed. He had previously tolerated quinoa without adverse reactions. However, the two last ingredients were obtained from a new brand. His physical exam was unremarkable. Percutaneous skin testing was positive to soy and prickprick testing to mustard seed. He was instructed on avoidance measures including food label reading and prescribed epinephrine auto injector. Conclusions: This case report demonstrates the importance of an adequate clinical history and detailed content of ingredients. Introduction: We present a case of a toddler with recurrent bacterial meningitis of unclear etiology, various upper respiratory infections, allergic rhinitis, and persistent cough who was eventually found to have a cerebral spinal fluid (CSF) leak. The case is interesting in that there were vague clues to her CSF leak over the years, but it was not until her second case of meningitis that prompted a cisternogram, which confirmed the diagnosis of a Mondini deformity. Case History: A 3 year-old female with a past history of bacterial menin-gitis and subsequent left-sided stroke, allergic rhinitis (based on positive skin testing), and asthma, who presented to the hospital for headaches and vomiting after two minor head traumas at daycare. Because of her history, CSF studies were obtained and revealed recurrent meningitis, which prompted further workup confirming the CSF leak. She was found to have a congenital cochlear malformation, which was then repaired by otolaryngology. Outcome & Discussion: Retrospectively, her history and various hospital visits were all consistent with CFS leakage. The patient actually presented to the hospital at 9 months of age after a minor fall with reported vomiting and lethargy. Afterwards, she was seen at various points for upper respiratory infections, allergic rhinitis, as well as meningitis with stroke, and asthma with hypoxia. She was also seen by an allergist/immunologist for workup of her food allergies and asthma. She was diagnosed with allergic rhinitis based on clinical history of rhinorrhea and mildly positive environmental skin testing. The rhinorrhea showed moderate improvement with intranasal corticosteroid treatments. During her first episode of meningitis, she had a basic immunological workup including immunoglobulin and complement levels and vaccine titers, which were all normal. It was not until her second episode of known meningitis that there were considerations for CSF leak as a source of her infections. Both instances of meningitis were only suspected because of acute neurological signs. Conclusion: Though rare, CSF leakage should always be considered in the differential for clear rhinorrhea and recurrent meningitis. In our case, there were several clues for CSF leakage including: history of head trauma, persistent clear rhinorrhea despite appropriate therapy for allergic rhinitis, and recurrent meningitis despite a normal immune evaluation. OMALIZUMAB MAY PREVENT PERFUME TRIGGERED LARYNGEAL ANGIOEDEMA. R.M. Young * , P. Ciminera, East Meadow, NY. Introduction: Omalizumab is a monoclonal antibody to IgE indicated for use in asthma but also used in other IgE mediated conditions i.e.Allergic Rhinitis and recently indicated for use in chronic urticaria. But could it have a role in irritant induced angioedema? Methods: N/A Case Report: This is a 16yr old female who had multiple hospitalizations for status asthmaticus x 2 yrs. Sinusitis, GERD, allegies to environment/foods were diagnosed as well as Paradoxical Vocal Cord (PVC) dysfunction by ENT via rhinoscopy. On exposure to dust, acid reflux, chlorine in a pool or indoor pollutants like perfumes, she'd develop nasal congestion, hoarseness, a barking cough, and throat tightness. She was placed on maximal medicines of steroid inhaler/LABA, montelukast, oral antihistamines, steroid nasal spray, pantoprazole, and oral steroids in an effort to control these symptoms. But since PVC was also a problem, speech therapy/counseling were started as well as immunotherapy (IT). Despite this regimen, when the patient was exposed to perfume in school, she required Epinephrine, Diphenhydramine, and Albuterol for symptoms of cough, throat closure and syncope with eventual admittance to the Pediatric ICU. This reaction was so severe that the child became home tutored due to the fear of perfume exposure. A similar reaction but milder to dust mite IT was witness and quickly reversed with Epinephrine and Diphenhydramine IM. The patient had an IgE level of 1033 IU/ml, so Omalizumab was started. For the past 1 year, she has received 375mg SQ every 2 weeks. She has returned to school and normal activities and hasn't had any symptoms requiring Epinephrine or Albuterol on exposure to perfumes. Conclusion: Since the patient was on medicines to control asthma, GERD, and sinusitis, it was thought that her throat closure was due to PVC triggered by the perfume. But Laryngeal angioedema seem to be the culprit. Irritants like perfume may have an IgE mediated process or a mechanism of destablizing mast cells similar to idiopathic anaphylaxis that hasn't been identified. Therefore, Omalizumab should be considered as a trial therapy. More studies are needed using omalizumab for these cases. Primary cilia dyskinesia (PCD) is an autosomal recessive genetic disorder characterized by recurrent and chronic infections of the upper and lower respiratory tract due to impaired mucociliary clearance. Patients with PCD can have co-existing asthma. Omalizumab is a high affinity recombinant humanized monoclonal anti-IgE antibody which is commonly used to treat uncontrolled moderate to severe allergic asthma. We report a case of 58 year old female with known diagnosis of PCD and persistent allergic asthma who presented with symptoms consistent with infections and expected course of PCD but were in fact due to uncontrolled asthma and successfully treated with Omalizumab. At the time of presentation, she was already on maximal therapy for airway clearance for her PCD. She had been treated with numerous courses of antibiotics for infection without improvement for the previous 5 years. At her initial visit, her pulmonary function test revealed component of obstruction with airway hyper-responsiveness that was reversible. Her physical exam revealed coarse breath sounds and wheezing that improved after bronchodilation. Despite PCD being her dominant disease pathology, she was thought to have a component of uncontrolled allergic asthma. As she was maximized on Beta 2 agonist and inhaled corticosteroid therapy, the decision was made to start Omalizumab given her elevated total serum IgE level. Within 3 months of starting Omalizumab, the patient reported significant improvement in her shortness of breath from baseline and her appetite. At the time of this report, approximately 2 years after starting Omalizumab, she has an overall improvement in FEV1, improved symptoms of shortness of breath, and decreased exercise induced bronchospasm symptoms. In summary, due to their motile cilia disorder, PCD patients have variable degrees of decline in their pulmonary function. While a majority of their respiratory symptoms are likely due to structural lung damage related to recurrent infections, other etiologies for shortness of breath such as concomitant allergic asthma should not be overlooked. In addition, the use of Omalizumab in the treatment of allergic asthma as addon therapy should be considered in patients with elevated total IgE level even in the setting of significant structural lung disease. F. Pazheri *1 , B. Schroer 2 , 1. Shaker Heights, OH; 2. Cleveland, OH. Background: Beetroot is a common food ingredient and are consumed all over the world. It is used as a food additive in many food products. Allergy to beetroot (Beta Vulgaris) is rare. Until now only one case of apparent anaphylaxis to beet root consumption has been reported (de Oliveira et al, Clinical and Translational Allergy 2011) ; there are a few reports of asthma and rhinoconjunctivitis induced by inhaling the vapor of cooked beet. Methods: We report a case of a 17 year old young man who had an anaphylactic reaction after eating beetroot. His medical history was notable for anaphylaxis to tree nuts, allergic rhinitis, Latex allergy and a subsequent diagnosis of Crohn's disease. The patient had oral and throat itching, vomiting, and a sensation of throat closing up within one minute after ingestion of one shot glass amount of a juice smoothie containing ¼ of a beet, carrots, and apples in 16 Oz glass of juice smoothie made at home. Symptoms slowly resolved over the next hour after 50 mg of diphenhydramine and monitoring in an urgent care clinic. He had never eaten beets before and there are no tree nuts in his home due to his tree nut allergy. He has safely eaten carrots and apples after the reaction. He was evaluated for possible allergy to beetroot with blood testing and skin prick testing. Results: Food specific IgE to beet root were positive at 2.52 KU/L. Skin prick test using fresh beetroot juice using a prick-prick method with a bifurcated needle on the forearm was positive with a 9 mm wheal and 20 mm flare (Negative control 0/0 mm wheal/flare, histamine 6/20 mm wheal/flare). Beet sugar prickprick skin testing and food challenge was negative. Patient has not had a subsequent allergic reaction to any food since the reaction. Conclusion: Anaphylaxis to beetroot is very rare and this is the first case reported with positive fresh food prick-prick testing that we could find. Refined beet sugar may not lead to reactions in beet allergic patients. Introduction: DRESS is a potentially life-threatening hypersensitivity reaction associated with a variety of medications, generally anti-epileptics. Patients present with rash, fever, hypereosinophilia, lymphadenopathy, and internal organ involvement, often hepatitis, usually within two months after initiation of a medication. Methods: Case report Results: A 50 year old African American Male with HIV (CD4=400), HCV Genotype 1a, and HTN, presenting with rash for one week prior to admission (PTA). Patient was in his usual state of stable health, with undetectable HIV viral load on HIV treatment, until 3 months PTA when he started Interferon gamma (IFN-g) for asymptomatic hepatitis C. One week post IFN-g therapy, patient developed dyspnea and fatigue. Two weeks PTA, patient self discontinued IFN-g and then one week PTA developed a rash. Patient noted a pruritic rash starting on his scrotum which he initially attributed to tinea cruris. He used nystatin powder, which temporarily relieved the itching however the rash began to spread to his bilateral lower extremities and then to his upper body. During the three months preceding admission, HIV regimen was also adjusted multiple times for increase in HIV viral load. Review of laboratory studies showed peripheral eosinophilia (absolute eosinophil count: 1.8 K/cumm), peripheral smear with atypical lymphocytosis, and stool examination for ova and parasites as well as serum testing for Strongyloides were negative. Patient suffered from acute renal insufficiency. Liver involvement was not detected. Patient was diagnosed with DRESS. Diagnostic criteria included fever, acute rash, hypereosinophilia, lymphadenopathy, and atypical lymphocytosis. DRESS was likely attributed to abacavir; however, other HIV medications or IFN-g could not be ruled out definitively as the cause. Patient required large doses of steroids to control symptoms and had an extensive hospital course. Post-mortem exam revealed cause of death to be gramnegative sepsis (Acinetobacter baumannii) without evidence of hepatic necrosis. Conclusion: In general, DRESS carries a mortality rate of 5-10%, which is in patients without co-morbid conditions. This case is unique because it illustrates patients with DRESS and underlying HIV maybe suspect to even higher mortality rates. Introduction: T helper1 (Th1) lymphocytes have important role in immune response against Tuberculosis(TB). Relationship between TB and high immunoglobulin E(Ig E) levels with Th1 related mechanism was told in recent studies and reduction in high Ig E levels was noted with TB therapy. Aim: To discuss relationship between high and persistant Ig E levels and TB. Case: Twelve-years-old female patient who have had chronic productive cough, frequent otitis and sinusitis since 6 months-old and was followed-up with asthma, resistant atelectasis of right middle lobe, bilateral bronchiectasis was admitted. Previous tests were normal except high IgE (494 mg/dl), TB history of her uncle 5 years ago. After admission, atelectasis was found in computarized tomography (CT) and bronchoscopy revealed dense purulent mucus. Aspergillus fumigatus and Hemophilus influenzae were observed in BAL culture. Symptoms persisted and total IgE increased (1750 mg/dl) despite appropriate therapy. Aspergillus fumigatus in prick test, Aspergillus spesific IgE, control sputum culture, and immune deficiency tests were negative. PPD, Quantiferon test, and ARB in gastric aspiratation were 20mm, positive and negative, respectively. Control CT revealed multiple intrathoracic conglomerated lymph-nodes (30x25 mm). Mediastinoscopic biopsy for Lyphoma resulted reactive hyperplasia. Anti-TB therapy was given to patient who had TB disease contact, PPD and quantiferon positivity, infiltration and conglomerated lymph-nodes resistant to therapy. Clinical and radiological improvement and reduction of IgE (289 mg/dl) were seen in second months of therapy. Conclusion: This case was presented due to rare presentation TB with high Ig E levels with almost complete improvement. EVALUATION OF EOSINOPHILIA: HYPEREOSINOPHILIC SYN-DROME. G. Rosner *1 , D.W. Rosenthal 2 , 1. New York, NY; 2. Great Neck, NY. Background: Eosinophilia can have multiple etiologies including atopy, parasitic infections and Churg-Strauss. Hypereosinophilic syndrome(HES) can be initiated by myeloproliferative and lymphocytic causes. Case Report: A 16 year old male was admitted for a 2-week history of new onset chest pain and shortness of breath. Four months prior to admission(PTA), the patient presented with transaminitis and peripheral eosinophilia to another academic medical center, where corticosteroids were tapered over 2 months. Two weeks PTA, the patient developed intermittent, severe, mid-sternal chest pain, shortness of breath, a racing heart, blurred vision, and dizziness, which progressively worsened as he developed bilateral joint pain. Results: Upon admission the patient had eosinophilia(3.9x10 9 /L), ESR 38, IgE 130, LDH 375. Results from the prior admission showed an ultrasound with hepatic inflammation, and a liver biopsy with fibrosis, and rare portal and lobular eosinophils(Eos). Esophageal biopsy revealed mild Eos infiltrates and bone marrow biopsy showed myeloid hyperplasia with increased Eos. Cytogenetic studies for FIP1L1-PDGFRA were negative. During this admission, transaminases, bilirubin, alkaline phosphatase, cardiac enzymes, ANCA, tryptase, vitamin B12 and IL-5 were all normal. CTA chest and cardiac MRI were unremarkable. Chest X-ray showed bibasilar small pleural effusions, spirometry showed restrictive lung disease, and bronchial biopsy had mild Eos infiltrate. Infectious disease studies were unremarkable. The patient was restarted on corticosteroids and Eos decreased(1.8x10 9 /L). As corticosteroids were tapered over weeks, chest pain recurred with associated increased Eos, C-reactive protein and LDH, which resolved with increased corticosteroids. Discussion: Multiple etiologies of eosinophilia were considered including infection, hematopoietic clonal eosinophilic proliferation, over-production of eosinophilic cytokines, Churg-Strauss and others. Symptoms were controlled with corticosteroids. The diagnosis of HES remains without a specific molecular or laboratory biomarker. Thus, it remains essential to diagnose and treat HES when patients present with peripheral hypereosinophilia(>1.5x 10 9 /L on 2 examinations separated in time by at least 1 month) with tissue hypereosinophilia(20% eosinophils on bone marrow biopsy, or significant eosinophilic tissue infiltration) and with organ damage or dysfunction. Introduction: Autosomal dominant hyper IgE syndrome (HIES) is a complex primary immunodeficiency disorder characterized by recurrent skin and sinopulmonary infections, eczematoid dermatitis, and elevated serum IgE levels. Disseminated infections by endemic dimorphic fungi have been reported to affect patients with HIES. However, invasive histoplasmosis rendering a catastrophic terminal ileum perforation has not been previously described. Case Description: We present the case of a 21-year-old man with HIES who developed recurrent episodes of diffuse abdominal pain, low-grade fever, intermittent emesis and unintentional weight loss. CT of the abdomen and pelvis on admission revealed partial small bowel obstruction. Laboratory findings demonstrated significantly elevated urine histoplasma antigen (15.82 ng/mL) and positive serum histoplasma antibody, implying a disseminated infection by H. capsulatum. He responded well to fluid resuscitation, GI decompression, and intravenous liposomal amphotericin B which was later transitioned to oral itraconazole 200 mg twice a day after the acute episode resolved. While he was maintained on itraconazole over the following year, the level of urine histoplasma antigen was assessed periodically. The level was trending down gradually but still remained detectable, likely due to his noncompliance to the antifungal therapy. The patient ultimately developed terminal ileum perforation which was successfully treated with partial small bowel resection and primary anastomosis. GMS staining of the resected small intestine revealed H. capsulatum within histiocytes. He recovered well after the surgery and continued the antifungal therapy. Discussion: In HIES, STAT-3 mutations result in an aberrant T H 17 cell response and impaired β-defensin production and neutrophil trafficking. These defects likely account for the susceptibility to invasive fungal infections. Ileocecal histoplasmosis may clinically mimic inflammatory bowel disease. Thus clinicians should be vigilant for the development of histoplamosis in HIES patients. Timely diagnosis and adequate antifungal therapy are crucial in preventing severe complications. Stings and bites are a common cause of allergic reactions. We report a case of anaphylaxis by Pogonomyrmex ant bite. It was diagnosed by clinical features and confirmed with skin tests. Currently in treatment with specific immunotherapy. Case Report: Fifty-six years old male. A year ago, while working in the garden home, he is bitten by an ant on his right hand presenting erythematous, pruritic confluent hives, swelling of eyelids, face and hands without cough or respiratory distress. He improved with oral antihistamines. Six months ago he suffers again an ant bite and besides the previous symptoms, he has nasal obstruction, rhinorrhea, fatigue. He received treatment with intramuscular antihistamine and steroids. A month ago, he is bitten a third time by an ant. He presented confluent pruritic erythematous rash, eyelids, mouth and hands edema, cough and dyspnea, within minutes he had loss of consciousness. He treated with oxygen, 0.5 ml intramuscular epinephrine, intravenous steroids and intramuscular antihistamines, intravenous fluids and improves gradually until he is discharged. Complementary studies show 7100 leukocytes with 59% neutrophils (4200), 30% lymphocytes (2200), 2.3% eosinophils (200), 0.4% basophils (4), blood chemistry, renal function tests and liver function tests are normal, nasal cytology with eosinophils, serum total IgE 1220 IU / ml. Skin tests were performed with the criteria AAAIC at a dilution of 1: 10,000 with a positive result for Pogonomyrmex harvester ants. Nonpharmacological therapy (information, "allergic to ant" bracelets and emergency actions) and pharmacological (epinephrine, steroids, antihistamines, and albuterol) was initiated. Immunotherapy starting at 1:1,000,000 dilution was indicated. He is currently asymptomatic and in a dose escalating immunotherapy protocol. Discussion: We show allergic reaction to Pogonomyrmex spp ant with positive intradermal skin test. Immunotherapy is safe so far. Although it is still not established the positive predictive value of a skin test, the combination of symptoms and skin test reaction indicated an IgE-mediated reaction. Pogonomyrmex allergy could be deadly. The immunological mechanism of anaphylaxis in this patient is caused by a type I hypersensitivity reaction. Background: Drug reaction with eosinophilia and systemic symptoms (DRESS) is a rare, possibly life-threatening drug reaction typically consisting of recent exposure to a high-risk medication (initiation weeks to months prior to symptoms) accompanied by skin eruption, systemic symptoms (fever, lymphadenopathy, hematologic abnormalities like eosinophilia) and end-organ involvement. Failure to recognize this disease, especially with a history of exposure to a high-risk medication, may lead to significant morbidity and mortality while removal of the offending agent can be lifesaving. Case: A 9 year-old African American male with a history of cerebral palsy, epilepsy, and GERD presented with a diffuse erythematous rash along with fever, cough and progressive dyspnea. His medications included phenobarbital and valproic acid started two weeks prior to presentation with long-term use of omeprazole. Family and social history were unremarkable. Initial physical exam was significant for a diffuse maculopapular rash without mucosal involvement and coarse breath sounds. His chest x-ray demonstrated patchy airspace opacities bilaterally. He was admitted to the hospital with continuation of his home medications and initiation of ampicillin for possible pneumonia. He clinically worsened. He required CPAP for respiratory support. He developed facial edema, eosinophilia (max AEC of 1560) and a transaminitis. His renal function remained normal. Differential diagnosis included drug reaction such as DRESS, infection or hypereosinophilic syndrome. His phenobarbital and valproic acid were discontinued four and six days later, respectively. He concomitantly received high-dose corticosteroids. His clinical status and laboratory findings returned to baseline with a slow corticosteroid taper over the next eight weeks. Conclusion: A history of high-risk medication use as well as clinical manifestations of skin eruption with systemic symptoms and end-organ involvement should prompt the possible diagnosis of DRESS. The most effective treatment is to remove the offending agent along with supportive treatment. The use of systemic corticosteroids is typically implored in patients with severe end-organ involvement. Laboratory abnormalities should be monitored closely until nor-malized. Importantly, the allergist should be familiar with and serve as a knowledgeable resource for the diagnosis and treatment of DRESS. Introduction: Idiopathic CD4 lymphocytopenia (ICL) is a rare disorder of unknown etiology. Diagnostic criteria include a persistent CD4 T cell lymphopenia with no underlying primary or secondary immunodeficiencies, with a CD4 T cell count < 300 cell/mL or a CD4 T cell count < 20% total lymphocytes on multiple occasions. Case Report: 52 year old Caucasian female, with history of SLE controlled on plaquinil, presented with a 2 month history of headaches associated with confusion, dizziness, nausea/vomiting, and right eye pain. Upon admission, an MRI revealed leptomeningeal enhancement. Cryptococcus neoformans grew on CSF culture. Amphotericin B and flucytosine were initiated. An absolute CD4 count was 86 (normal (N): >500). HIV testing with PCR, HSV, hepatitis panel, PPD, HTLV, and histoplasmosis and blastomycosis antigen tests were all negative. Immunology consult was obtained. B cell lymphocytes, NK cells, immunoglobulin levels, mitogen proliferation studies, CH50 and C2 were normal. Antibody titers for diphtheria, tetanus, and streptococcus pneumonia revealed protective levels. Based on the presentation, lab findings, and no identifiable cause for low CD4 count, the patient was diagnosed with ICL. She was discharged on amphotericin and flucytosine and prophylactic treatment with trimethoprim/sulfamethoxazole was initiated. The patient was medication compliant and asymptomatic at her 2 week follow-up visit. The repeat labs revealed an absolute CD4 and CD8 count of 37 and 91 (N: >200), and a CD4/CD8 ratio of 0.41. Discussion: Patients with ICL are usually identified when the CD4 count is less than 200 and complications develop. Opportunistic infections are the most common initial manifestations. Patients also present with autoimmune conditions or malignancies. The incidence of asymptomatic patients with ICL is unknown. Currently, there are no specific treatment guidelines. Therapies used to increase CD4 levels, IL-2, interferon-gamma, and IL-7 or allogeneic hematopoietic stem cell transplantation, have variable results. In children, immunoglobulin replacement is considered. Prophylactic treatments for opportunistic infections based on the CD4 cell count should be considered. Conclusion: ICL is a diagnosis of exclusion when there is no identifiable underlying cause for a low CD4 count. A thorough immune system evaluation is necessary when contemplating this diagnosis. SUCCESSFUL TREATMENT OF ACUTE URTICARIA WITH OMALIZUMAB. K. Patel * , R. Ten-Boquera, K. Gundling, San Francisco, CA. Introduction: Omalizumab is a monoclonal antibody that binds free IgE and is used in treatment of allergic asthma and chronic idiopathic urticaria. We report the first case of acute urticaria refractory to maximal conventional therapy that responded to omalizumab therapy. Methods: A case report and review of the literature. Data: A previously healthy 36 year old woman presented with an acute episode of facial edema and full body hives 24 hours after receiving new furniture in her home. She presented to the ED where she received epinephrine, solumedrol, diphenhydramine and ranitidine; she was discharged home after symptomatic improvement. The following day she had recurrent symptoms; she was treated similarly as her prior episode with overall improvement. She was discharged home on prednisone and loratadine. The next day she again had similar symptoms and was admitted for management. During the next 3 days of her admission, she received the following daily medications: 150 to 200 mg of diphenhydramine, 60mg prednisone, 150mg ranitidine, 25 to 50 mg hydroxyzine, 10 to 40 mg cetirizine, and 10 to 20 mg of montelukast. Despite this maximal therapy, she continued to have full body urticaria with facial and pedal edema. A skin biopsy confirmed acute urticaria. Due to ongoing severe symptoms, she was given omalizumab (300mg), and within 24 hours had rapid resolution of disease. A large panel of laboratory studies was negative, except for elevated levels of plasma histamine (3.6 ng/mL) and total tryptase (12 ng/mL), supporting mast cell mediator release. To further evaluate the furniture as a possible cause, fabric was ground with sterile water; the skin prick test was negative. Patch testing was also negative. Her medications were tapered over a month, and she continued to be symptom free at her 3 month follow up off of all medications. Conclusion: A trial of omalizumab for severe, refractory acute urticaria was associated with rapid resolution of disease. Although cause and effect is not definitive, the patient had not previously responded to five days of aggressive conventional therapies. Review of the literature indicates that this may be the first successful treatment of severe acute urticaria with omalizumab. The cause of this patient's outbreak remains unclear, but might be associated with chemicals used in the furniture, as indicated by other reports in the literature. A RARE CASE OF ACUTE COMPARTMENT SYNDROME SEC-ONDARY TO INSECT BITE. F. Farri *1 , T. Akande 2 , S. Baghian 2 , Y.K. Persaud 2 , 1. Ossining, NY; 2. Bronx, NY. Introduction: Acute compartment syndrome (ACS) is a surgical emergency, mostly seen as a rare but severe complication of trauma; however there are nontraumatic causes. ACS secondary to insect bite is very rare; to our knowledge, there is only one previously reported case of ACS due to a mosquito bite. Case Report: A 21-month old female with past medical history of recurrent insect bites presented with extensive swelling of the hand and forearm. She had a small pruritic bump on dorsum of the left hand from a witnessed flying insect bite a few days prior. Subsequently, she developed decreased movement of the left wrist and tenderness with passive extension of the digits. There was no improvement of the swelling with topical antibiotic ointment and oral antihistamine. X-rays of the wrist and forearm showed soft tissue swelling, but was negative for fractures and or dislocations. A diagnosis of Acute Compartement Syndrome was made and immediate surgical intervention was undertaken. Intra-operative findings showed maximally elevated pressures (45mmHg) in the adductor-pollicis compartment of the left upper limb. Surgical decompression of the affected area was performed with subsequent resolution of the pain and swelling. Diagnostic evaluation showed normal white blood cell counts with differential, complement levels, total tryptase levels, total serum IgE, and IgE to mosquito and house fly. Blood and wound cultures showed no growth during both episodes, she did however have significantly elevated serum histamine levels Discussion: Most reaction's to insect bites are non-allergic manifestations of the venom's toxic effect, and present as erythema, pain and swelling. Insect bite hypersensitivity, may be mediated by immunologic mechanisms (IgE-mediated or non-IgE-mediated venom allergy) but also by nonimmunologic mechanisms. An important question in the clinical management of the patient was: did an infectious process (i.e. cellulitis) contribute to the initial clinical presentation of ACS? The absence of fever, the short duration from time of bite to presentation, the absence of differential warmth, the normal white cell count as well as negative blood and wound cultures made a bacterial super-infection unlikely. The patient was placed on daily antihistamines and the parents were instructed to be vigilant in accessing immediate medical care during each subsequent reaction. R. Gupta *1 , M. Segal 2 , 1. Vernon Hills, IL; 2. Philadelphia, PA. Rationale: Fungal sinusitis is generally limited to immunocompromised hosts including those with hematological malignancies, diabetes, and chronic steroid use. The most responsible culprits include Aspergillus, Fusarium, Mucorales, and dermatiaceous molds. Here we present a young female found to have a rare fungal species causing maxillary sinusitis requiring long term fungal treatment. Methods: Computed Tomography of Sinuses, Magnetic Resonance Imaging of Head, and sinus biopsies with gram stain and culture. Results: Our patient has a longstanding history of allergic rhinitis and asthma with 3-4 hospital visits a year never requiring intubation. She presented with complaints of severe sinus symptoms and subsequently had a CT scan demonstrating sinusitis with bony erosion at the base of the skull. Endoscopic surgery and maxillary sinus biopsies revealed fungal species of Schizophyllum radiatum. Itraconazole treatment was started with frequent monitoring of QT-interval and drug levels, with microbiology confirming adequate susceptibility of treatment. Clinically her sinusitis continued to improve. She remained on Itraconazole for 6 months, with total resolution of symptoms. Conclusion: We believe this patient's case demonstrates two facets of rarity. One, our patient does not fit the typical demographic of those who acquire fungal sinusitis. Second, Schizophyllum radiatum is an unfamiliar species to cause infection and bony destruction. This case provides insight into evaluation of patients deemed minimal risk for fungal sinusitis and the rare although morbid complications of fungal sinusitis. Introduction: Granulomatous-lymphocytic interstitial lung disease (GLILD) is a non-infectious pulmonary complication of common variable immune deficiency (CVID) that can have a profound impact on the quality of life and survival of affected patients. Combination chemotherapy with rituximab and azathioprine has been previously shown to improve pulmonary function and imaging in this subgroup ofthese patients. We describe a case of CVID with GLILD treated with rituximab and oral mycophenolate mofetil that had a similar positive outcome. Case Description: A 37 year-old female with history of idiopathic thrombocytopenic purpura (ITP), initially presented to our clinic for evaluation of a chronic cough and shortness of breath. Pulmonary function testing showed a restrictive pattern, without evidence of obstruction or reversibility. Laboratory results were remarkable for profound hypogammaglobulinemia (IgG 75, IgA < 5, IgM 13) with normal numbers of T and B cells on flow cytometry. Computed tomography (CT) of the chest showed extensive interstitial disease, including innumerable bilateral pulmonary nodules, as well as multiple enlarged mediastinal lymph nodes and splenomegaly. She was started on monthly intravenous immunoglobulin (IVIG) therapy and underwent wedge biopsy of the lung for diagnostic purposes. Pathology reported follicular bronchiolitis, lymphocytic interstitial pneumonia, and non-necrotizing granulomatous inflammation as well as areas with fibrin deposition and organizing pneumonia. She was treated with 2 doses of rituximab (2g) two weeks apart and 1000mg of oral mycophenolate mofetil twice daily. The therapy was well tolerated. Six months following initiation of therapy, repeat imaging showed near resolution of pulmonary nodules with significant improvement in mediastinal lymphadenopathy and ground-glass opacities. Pulmonary function had returned to normal and the patient's cough and shortness of breath had resolved. Conclusions: Multiple authors have previously documented that monotherapy for treatment of GLILD in CVID is not effective. Combination chemotherapy using rituximab and azathioprine has previously been shown to be effective. This is the first case describing case describing successful treatment of GLILD with a combination of rituximab and mycophenolate mofetil. A.C. Netterville * , A. Ochoa, New Orleans, LA. Introduction: Mastocytic enterocolitis is a proposed term for cases of diarrhea-predominant irritable bowel syndrome (IBS) with elevated numbers of mucosal mast cells. GI mast cells excess is defined as the presence of greater than 20 mast cells per high-power field of microscopy in the GI tract mucosa. Presentation is chronic intractable diarrhea, often associated with abdominal pain with unremarkable colonic or duodenal biopsy specimens on routine hematoxylin-eosin staining, but increased numbers of mast cells on immunohistochemistry for mast cell tryptase. Case Description: This is a 6 year old male who initially presented at 23 months with chronic diarrhea and abdominal pain. He was evaluated by GI with an upper and lower endoscopy, and was found on immunohistochemical stains for CD117 to have greater than 20 mast cells per high power field. Elevated numbers were found throughout the colon and rectum. He had extensive additional evaluation which revealed normal total immunoglobulins, IgG subclasses, tryptase, C1 esterase inhibitor level and function, and complete blood count. He also had negative immunocaps to all foods and inhalant allergens tested. He was initially treated with hydroxyzine and singulair with little improvement. He was later started on gastrocrom four times a day, and at that time demonstrated significant improvement, with near resolution of his symptoms of diarrhea and abdominal pain. Upon evaluation by us we recommended adding an H2 receptor antagonist. Conclusion: Mast cells play an important role in the regulation of GI visceral sensitivity and vascular permeability. Increased numbers of GI mucosal mast cells has been documented in several studies in patients with irritable bowel syndrome, mastocytic enterocolitis, systemic mastocytosis, mast cell activation syndrome, and allergic mastocytic gastroenteritis and colitis. Patients with chronic unexplained diarrhea should undergo colonoscopic biopsies with special mast-cell stains. If elevated mast cells are demonstrated on biopsies patients should be treated with H1 and H2 receptor antagonist and also a mast cell stabilizing agent. In more severe cases it has been proposed to add an antileukotriene or a 5-lipoxygenase inhibitor. Introduction: Cow's milk protein allergy is common in infancy. About half of all cases are non-IgE-mediated, and allergic proctocolitis with bloody stools is considered the most benign form. Methods: Case Report. Results: A 4-monthold Hispanic female presented with complex febrile seizure and was incidentally noted to have grossly bloody stools. After a full-term delivery, she had been treated with prophylactic antibiotics due to maternal chorioamnionitis. During the NICU stay she was breastfed with cow's milk formula supplementation, and had intermittent heme-positive stools. Grossly bloody stools first occurred at 1 month of age, after which the mother eliminated cow's milk formula and began to exclusively breastfeed. Over the subsequent months, the patient had multiple episodes of grossly bloody stools after maternal dairy ingestion, but was otherwise thriving. Serum IgE to milk protein was negative. Review of laboratory studies showed peripheral eosinophilia (1.9k/mm 3 ) 4 days after birth and at time of presentation (3.3k/ mm 3 ). Stool examination for ova and parasites as well as serum testing for Strongyloides and Toxocara were negative. Given severe eosinophilia over a 4-month period beginning shortly after birth, screening was done for target organ damage. Urinalysis and liver tests were normal, but Brain Natriuretic Peptide (BNP) was elevated at 626 pg/mL, (no cardiac symptoms and echocardiogram normal). All dairy products were eliminated from the maternal diet, with subsequent resolution of eosinophilia (absolute eosinophils 100/ mm 3 ) and significant decrease in BNP (211 pg/mL). Conclusions: Milk protein allergy presenting with bloody stools is common and generally considered benign. The diagnosis is based upon clinical presentation and resolution of symptoms upon withdrawal of the presumed food antigen, and therefore labs are not routinely checked. However, this case illustrates that even intermittent exposure to cow's milk protein over several months may lead to persistent eosinophilia, which in other conditions has been shown to cause target organ damage. Elevated BNP levels have not previously been reported in hypereosinophilia. This case suggests that BNP may be a sensitive early marker for eosinophilic myocardial insult, although further studies would be needed to support this hypothesis. A.K. Wong * , C. Parrish, S. Thobani, M. Li, L. Scott, Los Angeles, CA. Introduction: Angioimmunoblastic T-cell Lymphoma (AITL) is a peripheral T-cell lymphoma which presents with non-specific systemic illness including lymphadenopathy, hepatosplenomegaly, B-symptoms, rash, and/or anemia. Rash is present in 20-60% of patients, and they are usually pruritic with lymphohistiocytic vasculitis seen on biopsy. Methods: Case Report Results: A 64 year-old Hispanic male with no prior medical history presented to an outside clinic with a 2-week history of generalized, erythematous, papular, nonblanching, pruritic rash over his trunk and extremities. After a 5-day steroid burst, the rash did not improve. 2 weeks later, he presented for evaluation. He was noted to have lymphadenopathy. Lymph node and bone marrow biopsies yielded a diagnosis of AITL. He received 1 round of chemotherapy with complete resolution of his rash. He was well for 2 weeks until he presented to the emergency room with fever of 101.3°F and neutropenia of 700/mm 3 . Cefepime and vancomycin were started on admission, and 2 days later he developed generalized facial swelling with a maculopapular erythematous rash over his trunk and extremities. The patient developed a fever of 101.8°F around the time of rash onset, and on physical exam, he was noted to have axillary and inguinal lymphadenopathy. Review of laboratory studies showed peripheral eosinophilia which increased from 0/mm 3 on admission to 1100/mm 3 on hospital day 7. Allergy and Immunology was consulted to evaluate for drug reaction with eosinophilia and systemic symptoms (DRESS). Conclusions: Allergists/Immunologists are commonly consulted for rashes, especially in relation to drug hypersensitivities. While DRESS is a potentially life-threatening adverse reaction to medications, the onset of DRESS is typically 2-8 weeks after initial exposure to the offending medication. The time course of this patient's rash was not consistent with DRESS. This case illustrates the importance of maintaining a broad differential diagnosis including infection or malignancy in the evaluation of a patient with rash of unknown origin. Generalized non-specific rash is a common presenting feature of AITL, and in this particular patient, his DRESS-like symptoms of fever, lymphadenopathy, and eosinophilia and systemic symptoms were explained by his underlying malignancy. His rash and DRESS-like symptoms improved after starting his second round of chemotherapy. Rationale: Dyshidrotic eczema is a known rare side effect of intravenous immunoglobulin (IVIG) therapy in patients who are most commonly treated for neurologic diseases. Its pathophysiology is not clear. Eczematous eruption has never been reported in patients treated with IVIG for immunodeficiency states, highlighting the role of high doses of IVIG in this peculiar side effect. The skin changes usually respond well to topical steroids and some patients may improve with continuation of the IVIG. Methods: Review of the literature and case report of a patient in which the use of Gamunex resulted in appropriate myasthenia gravis control but intolerable dyshidrotic eczema, which resolved when the formulation was switched to the Gammagard liquid form. Results: 37 year-old female with history of myasthenia gravis was started on Gamunex 2g/kg over 3 days, every six weeks. After the first infusion she developed dryness and scaling lesions over hands, scalp and chest which were diagnosed as dyshidrotic eczema. Patient did not have a history of eczema prior to receiving Gamunex. She was started on topical corticosteroids without much improvement in her skin lesions. Patch testing with common contact allergens and Gamunex was negative at 48 and 96 hours. Drug reaction was suspected and the patient's formulation of IVIG was switched from Gamunex to Gammagard 2g/kg over 3 days every 6 weeks. The skin changes remitted with the new product, but she developed 2 flairs of myasthenia gravis. The decision was made to switch back to Gamunex. Unfortunately, the patient's eczema reoccurred but her treatment with Gamunex was continued for optimum myasthenia gravis control. Conclusions: This is the first reported case of dyshidrotic eczema in a patient receiving Gamunex IVIG that did not improve with topical steroids but resolved after switching to an alternative formulation. It is unclear why the eczema improved with Gammagard. Both formulations use glycine and have similar osmolarity and content. Gamunex uses bromobutyl rubber stoppers in their vials while Gammagard liquid utilizes halobutyl or chlorobutyl rubber stoppers. Currently no comparative data exist describing the side effect profile in terms of appearance of dyshidrotic eczema among different IVIG formulations. H. Shah *1 , K. Gipson 2 , L. Wall 2 , 1. Yorba Linda, CA; 2. New Orleans, LA. Löffler syndrome is a hypersensitivity response to parasite larval migration through the lungs, and is rare in the modern United States. It is classically attributed to Ascariasis, but can occur with other parasites. A previously healthy 8year-old male was hospitalized with tachypnea, cough, and fever of one week's duration. History revealed exposure to pigs on his family's farm in southernmost Louisiana, no travel, no asthma or atopy, and normal review of systems. He was hypoxemic and imaging revealed diffuse reticulonodular lung opacities. He demonstrated leukocytosis, peripheral eosinophilia (39%), and high serum IgE (3,480 IU/mL). Given his acute respiratory failure, systemic corticosteroids were initiated. He was treated empirically for atypical pneumonia while undergoing diagnostic evaluation. Despite increasing serum IgE levels (peaking at 12,700 IU/mL), he demonstrated rapid clinical improvement and was weaned off of supplemental oxygen. Pulmonary function testing demonstrated a mixed obstructive and restrictive pattern. Broncho-alveolar lavage fluid showed profound pulmonary eosinophilia (86%). Subsequent evaluation of his eosinophilia revealed highly elevated serology to both Ascaris and Toxocara, which was treated with albendazole. Strongyloides serology was negative, as was an extensive evaluation for other infectious and allergic etiologies. This case demonstrates that Löffler syndrome may be severe, yet elusive. It may present with pulmonary involvement and fever without the involvement of other organs expected from larval migration, requiring a high degree of suspicion to make the diagnosis. One should consider it even in the absence of travel history, especially in endemic areas for Ascaris such as southeastern United States. Strikingly elevated eosinophils in the lung washings should prompt evaluation for parasites. Other aspects unique to our patient include living in close association with pigs and being seropositive to both Ascaris and Toxocara. Moreover, Ascaris suum is known to infect pigs and has rarely been reported with visceral larva migrans in humans. Systemic corticosteroids were effective in treating the patient's respiratory compromise, despite concern for dissemination of the parasite when host immunity is suppressed. Giant cell arteritis (GCA) is a chronic vasculitis of large and medium caliber blood vessels, mostly affecting individuals in the fifth decade of their life. It is caused by arterial wall inflammation that leads to the clinical manifestations of vasculitis and tissue ischemia such as jaw claudication, temporal headache and visual loss. Although it may be generalized, vessel inflammation most prominently involves the cranial branches of the arteries originating from the aortic arch. We present an uncommon manifestation of GCA in a patient with tongue swelling. Case Report: A previously healthy 68-year-old female was presenting submandibular swelling a month prior to evaluation. She was treated with antibiotics and intermittent doses of steroids for suspected sialadenitis. Despite therapy, the patient presented increased swelling of the tongue and pain with minimal headache, but had no blurred vision or jaw claudication. She was admitted to the hospital where a lingual biopsy revealed no amyloidosis or malignancies. Upon further testing, hereditary angioedema screening was negative but she still had elevated C-reactive Peptide (1.3 mg/dL). A trial of icatibant was administered for suspected bradykinin-induced angioedema. With no improvement of the symptoms, the patient was transferred to our institution for further evaluation. A biopsy of the temporal arteries revealed GCA and treatment was initiated with high-dose corticosteroids. Unfortunately, the necrosis of the tongue led to its eventual self-amputation. Conclusion: Lingual swelling and necrosis are extremely rare complications in GCA. That atypical presentation may mimic an angioedema episode when classic symptoms of GCA are subtle. This case reinforces the importance of suspecting GCA upon evaluating elderly individuals that present lingual swelling as to avoid a delay in diagnosis and treatment. We conducted a literature search review using Ovid. A search using the keywords "Giant Cell Arteritis and tongue necrosis" resulted in 43 published cases dating back to 1967. All cases were almost identical in clinical presentation to our case with the exception of some subtle differences. Six of the published GCA and lingual necrosis cases were triggered by ergotamine use. Also, the severity and extent of necrosis varied among cases, ranging from unilateral partial lingual necrosis to extensive necrosis of the tongue, scalp or bilateral vision loss. A. Asawa *1 , R. Bonds 2 , 1. Sugar Land, TX; 2. Galveston, TX. Introduction: Serum sickness is a type III hypersensitivity reaction caused by antigen-antibody complexes and presents with fever, urticaria, poly-arthralgia/poly-arthritis, and/or acute glomerulonephritis. In this type of reaction an antigen binds to antigen specific IgG forming immune complexes. These then bind to IgG receptors on inflammatory cells and activate complement causing an inflammatory reaction. Methods: We report the case of a 44 year old female referred to Allergy/Immunology clinic for hives and arthralgias. Patient first noted symptoms 4 years ago after she was treated with trimethoprim-sulfamethoxazole and mupirocin for cellulitis of her right foot. Two weeks later she noted diffuse erythematous, well defined, burning, circular skin lesions that blanched. She also had swelling and increased warmth in both knees and arthralgias in her knees, neck, elbows, and hands. Numerous antihistamines were tried, but not helpful. The symptoms gradually resolved in a few months. She was asymptomatic for four years. In April 2014 she treated blisters at the base of her right foot with mupirocin. One day later she noted a return of her diffuse urticaria, arthralgias, and fever. She was treated with a corticosteroid injection which did not alleviate her symptoms. Subsequently, she was started on cyclosporine which has provided her with moderate relief of symptoms. Discussion: The patient's history of repeat episodes of arthralgias, fever, urticarial rash triggered by a medication raises concern for serum sickness versus drug induced lupus. Laboratory evaluation revealed normal levels of C3 (123 mg/dl; reference range 86-184 mg/dl) and C4 (26.5 mg/dl reference range 20.0-59.0 mg/dl), elevated C1Q binding (4.7 ugE/mL; reference range 0.0 -3.9 ugE/mL), and ESR (45 mm/hr; reference rage 0-20 mm/hr) suggesting evidence of circulating immune complexes supporting the diagnosis of serum sickness over drug induced lupus. In our literature search, we did not identify any previous reports of mupirocin causing serum sickness. Conclusion: We report a 44 year old female old presenting with elevated C1Q binding and ESR along with recurrent fevers, arthralgias, and urticarial rash most likely secondary to serum sickness induced by mupirocin. Introduction: Bronchiectasis can occur in children due to cystic fibrosis, primary ciliary dyskinesia, infections, immunodeficiency, and anatomic etiologies. Primary symptoms include productive cough, chest pain, dyspnea, and fatigue. High-resolution computed tomography (HRCT) is the diagnostic imaging modality of choice. Management involves treatment of the underlying disease process, mucociliary clearance, and treatment of infections. Untreated, bronchiectasis causes worsening inflammation, airway obstruction, morbidity and mortality. Methods: We review a case of an adolescent female who presented with uncontrolled asthma and seasonal allergies to the Allergy/Immunology Division. Results: A 17 year old female presented with "uncontrolled asthma" and seasonal allergies. She had transient tachypnea of the newborn and RSV bronchiolitis at 2 months of age. At 1 year of age, she had her first episode of pneumonia. She then had pneumonia about once a year, requiring antibiotics. She had required high dose inhaled steroids and frequent oral steroid courses for asthma. She reported wheezing when recumbent and a feeling of fullness in her left lung with inability to sleep on her left side. Her examination was significant for crackles and inspiratory wheeze with dullness to percussion at the left lung base. Spirometry demonstrated reversible airway obstruction. Skin prick tests were positive to pollens. Sweat test was negative and immunologic evaluation demonstrated increase in antibody levels to pneumococcus following booster vaccination. Chest CT scan showed bronchiectasis in the left lower lobe. Bronchoscopy demonstrated narrowing of the orifice of the left lower lobe due to collapsibility of the posterior wall of the bronchus and thick, white secretions within the left lower lobe. Biopsy of the bronchus demonstrated chronic inflammation with ciliary disorientation. She was diagnosed with impaired mucociliary clearance and retained secretions due to left lower lobe bronchomalacia. She started an airway clearance regimen with significant clinical improvement and capacity to wean asthma therapy. Conclusion: In an asthmatic with wheezing, chronic cough, sputum production and focal findings on examination, bronchiectasis should be considered. Missed diagnosis, can create undue burden of disease and significant sequelae from excess asthma therapy. 51 year-old female suffered from recurrent lip, tongue and laryngeal angioedema for almost 2 years and she required two tracheostomies. Her daughter had a history of lip swelling. Laboratory reports: C4 undetectable, C1 esterase low, C1 esterase function was 40% and C1q normal. She was diagnosed with HAE. Danazol was started. Because of persistent cramping abdominal pain, and because she was going to start danazol, a CT of the abdomen was obtained; it showed mild splenomegaly. She was then evaluated by the oncologist who entertained the possibility of hereditary versus acquired angioedema (AAE). A bone marrow biopsy revealed the presence of a small CD5 negative, CD10 negative, CD19 & CD 20 positive Lambda restricted B-cell population. The pathologist felt that the presence of a small abnormal monoclonal B-cell population was suspicious but not definitive for the diagnosis of lymphomatous involvement of the bone marrow. Given the non-definitive bone marrow diag-nosis, the oncologist and the patient decided to observe with close follow up. Despite danazol, she suffered from continued life threatening attacks of angioedema. Two months later, C1 Esterase Inhibitor (Cinryze) infusion therapy was started in an attempt to prevent episodes of angioedema. By this time, her spleen was found be palpably enlarged. Repeat complement studies showed C1q to be low, in contrast to a normal C1q previously. After 2 months of Cinryze therapy, the patient underwent a splenectomy for Non-Hodgkin's lymphoma. During the period she received Cinryze, she did not have angioedema. One month post splenectomy, Cinryze was stopped. She continued to be free from episodes of angioedema. Four months later, C4 and C1q remained depressed, but C1INH function recovered nicely. This patient had an initial diagnosis of HAE. Later, the low C1q and palpably enlarged spleen prompted a diagnosis of AAE secondary to non-Hodgkin's lymphoma. This was an unusual presentation with an initially normal C1q in this patient with AAE. Cinryze therapy is not approved for use in acquired angioedema but was necessary in this patient with life threatening episodes. Cinryze therapy prevented further episodes of life-threatening angioedema until definitive therapy with a splenectomy was performed. Our patient has been completely free of angioedema for more than one year after splenectomy and discontinuation of cinryze therapy. Introduction: Orofacial granulomatosis (OFG) is a rare, disfiguring illness of unknown etiology characterized by the development of non-caseating granulomas in the oral and maxillofacial area. OFG can manifest as Melkersson-Rosenthal syndrome, which is a triad of persistent lip or facial swelling, recurrent facial paralysis and fissured tongue, or as cheilitis granulomatosa of Miescher, which primarily affects the lips. Although OFG can be associated with systemic diseases such as tuberculosis, Crohn's disease and sarcoidosis, it has not been reported with hereditary angioedema (HAE). Case: A 43-year-old woman presented with complaints of recurrent swelling of the face, lips, uvula, neck, tongue and limbs. The episodes occurred twice weekly and resolved over 2-4 days. High-dose antihistamines were not beneficial. Laboratory work-up revealed normal C4 level and normal C1 inhibitor level and function. She was diagnosed with HAE with normal C1 inhibitor level and successfully treated with C1 inhibitor concentrate, ecallantide and icatibant. However, six months after her initial presentation, the episodic lip swelling progressed to persistent lip swelling despite resolution of other symptoms. Physical exam revealed diffusely edematous upper and lower lips with mild overlying maceration. A lip biopsy demonstrated diffuse, submucosal infiltrate of lymphocytes, histiocytes, and multinucleated giant cells forming non-caseating granulomas, as well as negative Congo red stain, Periodic Acid-Schiff stain, and Fite stain. These findings were consistent with cheilitis granulomatosa. Oral and intravenous corticosteroids led to only mild temporary improvement, while dietary modification and topical corticosteroids were ineffective. The patient is scheduled to undergo intralesional corticosteroid injections. If these are unsuccessful, the next management consideration would be topical and/or systemic immunosuppressants. Conclusion: Orofacial granulomatosis is rare illness that can significantly affect patients' quality of life. Our case describes a unique presentation of OFG in the setting of HAE. Delay in recognition and diagnosis can result in permanent disfigurement and impairment in eating and drinking. Therefore, it is imperative for clinicians to consider OFG in their differential diagnosis for orofacial edema, and recognize that it can develop in the setting of HAE. Introduction: Acute antibody mediated rejection (AMR) is characterized by graft dysfunction manifesting over months or years. It is a result of donor specific antibodies that usually develop de novo after transplantation. A diagnosis of AMR typically indicates an increased incidence of allograft dysfunction and mortality. IVIG has been cited in previous studies as being used successfully to treat AMR in cardiac transplant patients. However, there are currently no studies that investigate the use of SCIG in the management of acute AMR, which has shown in this case to be a promising treatment. Case Description: Patient is a 68 year old female who is currently being treated for hypogammaglobulinemia (HGG) and acute rejection s/p cardiac transplant in 2008. Multiple biopsies of the endomyocardium post-transplant from 2008-2009 showed evidence of Grade 1A and 1B rejection. In 2010, the patient was found to have multiple grade 3A rejections, and received 3 days of IV Solu-Medrol and 7days of Thymoglobulin for presumed T-cell mediated acute rejection. She was subsequently admitted for photopharesis for 2 days and plasmapheresis with concomitant IVIG for 3 days the following month. In March 2013, the patient was admitted for another episode of 3A rejection, at which time she was also found to have low Ig levels: IgG 326, IgA 119, IgM 41, and IgE 1.9 with increased BUN/Cr. Patient was given IVIG, 1 gm/kg x 2 days. Subsequent biopsy revealed grade 1B rejection, and the patient had much improvement in her IgG levels. Patient was continued on monthly subcutaneous immunoglobulins (SCIG) at 500 mg/kg for treatment of acute antibody mediated rejection and HGG. She continued to maintain IgG levels within normal range in spite of end stage renal disease. Her subsequent pathology specimen reports, with the most recent from 4/2014, show grade 1B rejection, which is much improved from previous 3A rejections. Discussion: This case illustrates the potential benefit of using SCIG as part of the management for recurrent AMR. Early institution of this form of treatment could reduce the incidence of AMR, and therefore, prevent its complications including allograft dysfunction and mortality. Research on this topic is warranted. M. Gogna * , Glenshaw, PA. Kabuki syndrome (KS) is a multiple malformation/mental retardation syndrome with an estimated frequency of 1/32,000 that is characterized by distinctive facial features, skeletal anomalies, short stature, and mental retardation. Increased infections especially otitis media, URIs, and Pneumonia have been identified in 60-70%patients. It is unclear if these infections are due to Immune problems (Hypogammaglobulinemia is a frequent finding in Kabuki syndrome) or craniofacial abnormalities. We present the case of a 15 year old female with Kabuki syndrome who presented with persistently low B cells and Autoimmune enteropathy. Both these findings have previously not been reported in KS. Her Immunoglobulins have been trending down since last 5 years (table 1) Our patient gets mostly line infections with MSSA, CONS, Ecoli and Klebsiella and not sinopulmonary infections. This can possibly be explained by a disruption in skin barrier and disruption in GI barrier(h/o chronic diarrhea). Her Memory B cells and Mitogen assays were wnl which is very reassuring. She had significantly low B cells over the course of atleast 5years. Low B cells cannot be explained by chronic diarrhea or use of Tacrolimus ( started on Tacrolimus for Autoimmune-enteropathy). She was diagnosed with Autoimmune enteropathy based on the following 1.Inflammation and increased intraepithelial lymphocytes and numerous apoptoses on biopsy and 2.positive anti-enterocyte antibody and 3. anal fissures on exam. Recommendations: We recommend that a basic Immunological work up -quantitative Immunoglobulins, Specific antibody titers be done in all patients with KS. If the Initial work up is abnormal, we recommend further work up including Lymphocyte subsets, Mitogen assays and B CELL panel. An index of suspicion should be kept for Autoimmune enteropathy leading to Chronic Diarrhea. Background: Hashimoto's encephalopathy is a rare and likely under recognized immune-mediated neurologic syndrome. While the exact pathophysiology remains unclear, the encephalopathy itself appears to be independent of the biochemical thyroid status. Due to the rarity of the syndrome, minimal evidence is available to guide treatment decisions. The mainstay of treatment is corticosteroids, with some patients requiring other immunosuppressive agents. Cases of success with use of IVIG have been published. Few case reports exist in the literature of successful treatment involving thyroidectomy, with the rational of removing the thyroid as an antigenic stimulus. Case Presentation: We report a case of a 39 year old man who initially presented with a goiter and non-specific symptoms of dizziness, palpitations and general malaise. He was found to be hypothyroid and started on replacement therapy. He subsequently developed ataxia, thought to be cerebellar in nature with head titubation, gait dysfunction, cognitive impairment and inappropriate emotional responses. The severity of his symptoms caused him to be bed-bound. He had a reassuring MRI head, LP, and CT of his chest, abdomen and pelvis as well as a negative paraneoplastic antibody work-up. His anti-thyroid peroxidase antibodies and anti-thyroglobulin antibodies were significantly elevated. The treatment regimen initiated included high dose corticosteroids, with a prolonged taper as well as IVIG, addition of mycophenolic acid and thyroidectomy. Interestingly, pathology from the thyroidectomy revealed micropapilary cancer with local micrometastases in the lymph nodes. The patient returned to his baseline status, and presently is continued on monthly IVIG, mycophenolic acid and a continued prednisone taper. He has not had recurrence of his symptoms. Conclusion: The above case highlights a rare and likely under-diagnosed immunologic syndrome for which little guidance in the literature exists with respect to treatment beyond corticosteroids. This case report serves to review the clinical presentation, pathogenic theories, diagnosis and management options for Hashimoto's encephalopathy. The case also adds support to previous cases of successful treatment with thyroidectomy. Introduction: A twenty-four-year-old male was admitted to the ICU with deteriorating mental status and cogwheel movements. Two healthy individuals who accompanied the patient on a recent trip to Wyoming had spontaneous resolution of similar prodromal symptoms after exposure to mosquito bites. He was subsequently found to have neuroinvasive West Nile virus infection with positive IgM antibodies in his CSF and serum, and detectable serum RNA. The patient had a history of recurrent sinopulmonary infections, chronic warts, and leukopenia with monocytopenia. His father and grandfather died before 50 years of age due to viral illnesses. There was no known family history of leukemia or nontuberculous mycobacterial infections. Methods: Peripheral blood was analyzed by flow cytometry. NK cell function was measured by the release of radioactive chromium from lysis of the erythroleukemia cell line, K562. DNA amplification of all exons, flanking splice sites, and intronic noncoding regulatory regions of GATA2 was performed on whole blood. The patient received 1.2 g/kg IVIG divided over 3 days and 3 million units of subcutaneous interferon alfa-2b daily for 14 days. Results: Monocytes (0), B lymphocytes (8), CD4+ and CD8+ T lymphocytes (161 and 68), and NK cells (13) (/mL) were low. Absolute CD16+56+ and CD56 bright NK cell counts were markedly reduced, with relative frequency within normal limits. NK cell function was normal. Genomic sequencing revealed a heterozygous single base deletion, c.1021delG; p.A341Pfs, causing the loss of the highly conserved second zinc finger of GATA2. DNA sequencing for the same mutation in his mother was negative. The patient had a significant improvement in his neurologic function and most of his warts subsided after IVIG and interferon alfa-2b. He was referred to our Hematopoietic Stem Cell Transplantation Program for bone marrow aspiration/biopsy and evaluation for bone marrow transplantation. Conclusions: This is the first report of a patient with GATA2 deficiency presenting with West Nile virus encephalitis. Patients with heterozygous GATA2 mutations may be more susceptible to neuroinvasive disease from West Nile virus infection, even in the absence of a nontuberculous mycobacterial infection or familial history of leukemia. IVIG and interferon alfa-2b may have been beneficial for this patient with impaired viral immunity. Introduction: Erythropoietin replacement is a key therapy of anemia associated with chronic kidney disease. Rapid desensitization has been successful in patients with immediate type I hypersensitivity to epoetin alfa, and a slower two-day protocol has been described in an adult patient who had delayed type hypersensitivity reactions to the same agent. An eleven-year-old girl with a history of end stage focal segmental glomerulosclerosis on hemodialysis developed a diffuse, pruritic, maculopapular rash one day after starting epoetin alfa, which recurred when darbepoetin alfa was subsequently administered. She then failed a two-day epoetin alfa desensitization protocol but subsequently tolerated epoetin alfa after a seventeen-day desensitization. Methods: Binding and neutralizing anti-epoetin alfa and anti-darbepoetin alfa antibodies were measured using biosensor immunoassay (GE Healthcare-Biocare, Uppsala, Sweden). A two-day subcutaneous epoetin alfa (Epogen, Amgen) desensitization schedule, adjusted by weight, was modeled after a previously published adult epoetin alfa desensitization protocol. The patient developed an intensely pruritic, maculopapular rash prior to the first dose of day two. She was started on prednisone 0.3 mg/kg (10 mg) daily and a prolonged protocol was designed. Results: Anti-epoetin alfa and anti-darbepoetin alfa antibodies were absent. On day 4, the patient's rash resolved, and desensitization was resumed. She did not have pruritic rashes prior to subsequent doses. On day 17, she reached her target dose of 700 IU, and the prednisone was discontinued. At her three-month follow-up visit, she continued to tolerate the epoetin alfa without adverse reaction. Conclusions: To our knowledge, this is the first epoetin alfa desensitization for a child with delayed type hypersensitivity who experienced an adverse reaction to a two-day regimen. Subsequently, a slower dose escalation (seventeen days), with the support of a low dose systemic steroid, was successful at building a desensitized state to epoetin alfa. This case corroborates the princi-ple that slower desensitization may increase tolerability in delayed type hypersensitivity reactions. Rationale: Contact dermatitis (CD) is one of the most common inflammatory skin diseases diagnosed by allergists, dermatologists, and primary care providers. The presentation of CD is variable based on its time course; it can range from red clustered vesicles to bullae with both scaling and pruritus. Allergic CD can complicate or co-exist in the patient with atopic dermatitis (AD). Methods: An 11 year old female with a history of AD was referred to the Allergy/Immunology Clinic at Winthrop University Hospital for evaluation of persistent chelitis despite evaluation and management by her primary care physician and dermatologist. Over the last four years, the patient reported worsening of perioral and lip lesions. She had been treated with topical tacrolimus and corticosteroids with minimal improvement. She denied any perioral pruritis, worsening of lesions with food ingestion, or use of any cosmetic or lip balm products. She reported using the same toothpaste on a regular basis and denied licking her lips. She reported skin irritation with costume jewelry and hobbies included flute playing. Physical examination revealed dyshidrosis and chelitis (dry cracked lips and perioral erythema with no other active eczematous lesions present). Suspicion for allergic CD exacerbating atopic dermatitis. The patient was treated with emollients and continued use of a low potency topical CS. Results: The patient underwent patch testing with the North American Contact Dermatitis panel which included a total of 69 allergens including metals, cosmetics, fragrances, preservatives and medications. She had positive results to nickel (3+), cobalt (2+), and neomycin(2+). The nickel and cobalt were determined to be of probable relevance as they were likely components of her flute. The neomycin was considered to be of unknown or past relevance. She was counseled on a trial avoidance of her metallic flute. Conclusions: Nickel remains the most common allergen obtained from patch testing. In patients with lip dermatitis one must consider allergens including lip balms, lipsticks, toothpastes, gums and hobbies including playing an instrument. In patients with co-existing AD consider allergic CD, if there is not a response to medical pharmacotherapy and interventions, identification and avoidance of relevant allergens is the mainstay treatment of CD. Background: Antithymocyte globulin (ATG) is a lymphocyte-selective immunosuppressant indicated for the management of allograft rejection in renal transplant patients and aplastic anemia. ATG has the potential for adverse reactions including type I hypersensitivity reactions as well as other more com-mon adverse reactions including fever, chills, rash, nausea, arthralgia, headache, and phlebitis. Skin testing evaluates the potential for a type I hypersensitivity reaction. Despite a positive skin test, there are circumstances in which receiving ATG is deemed necessary and in such circumstances, ATG desensitization is recommended. Previous ATG desensitizations in adults have been unsuccessful. Clinical Vignette: The patient was a 43 year old male with aplastic anemia and mild intermittent asthma. His treatment started with cyclosporine and ATG. On the first dose of ATG, he developed dyspnea, fevers, hypotension, and tachycardia, which were treated with infusion cessation, acetaminophen, morphine, and hydrocortisone, with relief of his symptoms. A second infusion was attempted the next day per his initial protocol and again symptoms developed including dyspnea, tachypnea, chest tightness, fevers, and hypotension. The infusion was stopped, supplemental oxygen given, and a CT chest with contrast was obtained, which showed bilateral scattered ground glass opacities with a few areas of focal consolidation. Given the possibility of ATG-induced lung toxicity, no further ATG was given and the patient was maintained on cyclosporine. Eighteen months later, the patient was found to have worsening pancytopenia despite therapeutic cyclosporine serum levels. Given failure of treatment with cyclosporine, treatment with ATG was deemed necessary. As skin testing results would not have changed the plan for desensitization, skin testing was deferred. The patient underwent the ATG desensitization protocol outlined in Table I which was based on the protocol put forth by Ferdman and colleagues. He tolerated the protocol well without symptoms except fever on day 2 of the protocol which resolved with acetaminophen. Conclusion: Successful desensitization to ATG has been documented in only pediatric patients as adult patients have developed symptoms necessitating cessation of the desensitization. This case illustrates a successful desensitization to ATG in an adult patient with aplastic anemia. Introduction: Adrenal suppression and Cushing's syndrome have been reported due to the interaction between ritonavir and intranasal fluticasone. We report the successful use of an alternative intranasal steroid (INS) in a patient who previously had adrenal suppression due to ritonavir and intranasal fluticasone. Methods: Case report. Data: A 50 yo M with HIV on a boosting dose of ritonavir 100 mg BID as well as emtricitabine-tenofavir, raltegravir, and darunavir was treated with fluticasone intranasal spray 2 sprays per nostril daily for allergic rhinitis. After 6 months, he reported good control of rhinitis and lacked signs or symptoms of adrenal suppression. Three months later he was noted to have abdominal striae and easy bruising. Early morning cortisol was <1.0 and ACTH was found to be <5. He was started on a tapering dose of hydrocortisone and intranasal fluticasone was discontinued. Upon further inquiry, on some occasions the patient self-increased his INS dose up to 2 sprays tid. After completion of the hydrocortisone taper, early morning cortisol and ACTH were within normal range and his Cushingoid appearance resolved. After 9 months of treatment with intranasal antihistamines, oral antihistamines, as well as leukotriene antagonist therapy, his rhinitis symptoms were still not controlled and were greatly affecting his quality of life. A baseline morning cortisol and ACTH were normal. Intranasal mometasone was prescribed and he was counseled on the strict usage of mometasone 1 spray per nostril daily. After 4 weeks of continuous therapy, early morning cortisol and ACTH remained normal. After 6 months of follow-up, he continues to have excellent symptom control and absence of Cushing's syndrome on repeat examinations. Conclusion: Our case demonstrates that adrenal suppression with the concomitant use of intranasal fluticasone and ritonavir can also occur while on the lower boosting dose of ritonavir. This case also suggests that intranasal mometasone is safe and effective with concomitant ritonavir 100 mg twice a day in a patient with HIV. Background: We will describe two unique cases regarding the potential effects of allergy immunotherapy (IT) on eosinophilic esophagitis (EE). Methods: Patients underwent a complete history, physical exam, skin prick testing (SPT) by classical method, and an accelerated IT program. Both patients reached their maintenance doses, which was 0.5ml of a 1:1 concentration. EE was diagnosed via EGD and biopsy. Results: Case 1: Patient presented for evaluation of seasonal allergy symptoms and food allergies. Patient had a known history of EE that was previously controlled with an elimination diet. SPT revealed positive results to dust mites, cat, dog, grass, trees, weeds, milk, egg, peanut, multiple tree nuts, and sesame. Patient was started on IT for allergic rhinitis, and after six months of treatment the patient reported that dysphagia had progressively worsened despite avoidance of all known food triggers. Case 2: Patient presented with complaints of dysphagia and seasonal allergy symptoms. SPT revealed positive results to trees, grass, weeds and cockroach. Daily symptoms of dysphagia were uncontrolled on a proton pump inhibitor alone. The patient was referred to a GI specialist for a definitive diagnosis, and EE was confirmed with an EGD and biopsy. An elimination diet was not initiated. Patient was prescribed a daily swallowed budesonide and Splenda mixture for three months after which treatment was stopped with improvement in symptoms. Three months after swallowed budesonide was discontinued, allergy IT was initiated for treatment of allergic rhinitis. IT was continued for 1.5 years until discontinued by the patient. Upon completion, the patient reported that dysphagia had continued to improve since starting IT. Conclusions: The inhalation of aeroallergens may have an effect on the inflammation occurring in the esophagus of EE patients. It is hypothesized that using IT to reduce sensitization would greatly impact this inflammatory response, however, there are no controlled studies on allergy IT as an adjunctive treatment for EE. Our case reports indicate that there is conflicting data on whether or not IT can impact EE, and if this impact is harmful or beneficial. Our recommendations are to continue research on the role of aeroallergens in the pathogenesis of EE, to consider the effects of IT on EE, and to assess the risks and benefits of continuing IT on patients with EE. K. Anthony * , L. Wild, New Orleans, LA. Introduction: Chronic abdominal pain associated with bowel dysfunction affects 15% of the general population. These patients often undergo extensive negative work-ups and are diagnosed with functional pain disorders. Gastrointestinal mast cell disorders are not always considered in the differential diagnosis; however, recent studies suggest patients with chronic abdominal pain may have an underlying gastrointestinal mast cell disorder that will improve with appropriate therapy. We present a case suggesting these patients should be evaluated for a gastrointestinal mast cell disorder. Methods: A 32 y.o. male with allergic rhinitis presented with a 3 year history of diffuse abdominal pain, diarrhea, and a sensation of "itching" inside the abdomen. He denied any associated flushing, rash, wheezing, difficulty breathing, syncope, or dizziness. He had undergone an extensive evaluation including endoscopy, colonoscopy, CT abd/pelvis, CBC, CMP, ESR, and celiac screening. Given his persistent symptoms, he underwent a repeat endoscopy/colonoscopy with biopsies and staining for CD 117+ cells. Results: CT abd/pelvis, CBC, CMP, ESR, and celiac screening were normal. Previous endoscopy and colonoscopy were negative. Repeat endoscopy and colonoscopy with staining for CD 117+ cells was significant for >20-40 mast cells per hpf in the stomach, duodenum, terminal ileum, and colon. Systemic mastocytosis was ruled out with normal serum tryptase and normal bone marrow biopsy. He was started on daily H1 and H2 blockers with significant improvement in abdominal pain and diarrhea. Conclusion: Chronic abdominal pain and diarrhea in this patient were likely secondary to mast cell gastroenterocolitis. Treatments for mast cell gastrointestinal disorders including oral antihistamines and oral mast cell stabilizers are available and usually effective. Accurate diagnosis enables patients to start appropriate treatment, leading to improved symptom control and quality of life. This case suggests patients with persistent abdominal pain and bowel dysfunction may need evaluation for a mast cell GI disorder, even in the absence of symptoms of systemic mastocytosis. P. Uong *1 , K. Christine 1 , D. Wong 2 , 1. Phoenix, AZ; 2. Mesa, AZ. Introduction: We teach patient with allergies to avoid allergen as the first step in treatment, but what if that allergen is a life-saving treatment? We present a case of a child with severe hemophilia B who developed anaphylaxis to coagulation factor IX with subsequent desensitization attempts and associated medical complications. Case: TL is a 15 year-old male with severe hemophilia B who presented to the hospital for severe right arm pain and swelling, found to be compartment syndrome requiring an urgent fasciotomy. Despite his ill-ness, TL was not on coagulation factor IX, the one protein that he was missing, because of multiple instances of anaphylaxis and inhibitors to it. Interestingly, he had undergone two desensitizations as a toddler with eventual fair control of his bleeding despite high inhibitor levels. However, in recent years, allergic symptoms to factor IX started to escalate, requiring change of therapy to factor VII, which bypassed the factor IX-dependent step in the coagulation cascade and kept his bleeding under variable control. Outcome: TL continues to struggle with allergies to factor IX, acute on chronic pain exacerbations, and ongoing complications including a recent large deep venous thrombosis (DVT) in his left arm. He is now undergoing desensitization to factor IX again but is now using Rixubis instead of BeneFIX, with the addition of rituximab. Because the protocol by Sarah Alexander was denied by insurance, TL is now desensitizing according to the protocol by Dioun et. al at Boston's Children's Hospital. Advantages for rituximab include lowering inhibitor levels and bleeding symptoms with a reported beneficial change in factor kinetics. The disadvantage, however, is that rituximab can be associated with anaphylaxis, and in rare cases, multifocal leukoencephalopathy. With his debilitating disorder, depression is certainly a concern, but TL is now doing better than ever before, and hope exists for even newer therapies on the horizon, including several longacting formulations of factor IX. Conclusion: Hemophilia B is a chronic, lifethreatening, debilitating condition, but with a history of allergies and anaphylaxis to the one protein that is missing, treatment and management becomes much more complex. This case report is an important reminder of the need for a strong interdisciplinary team to stay involved and to stay abreast of new medical advances to help maximize care for this patient. P. Uong *1 , N. Jain 2 , 1. Phoenix, AZ; 2. Gilbert, AZ. Introduction: Defects in the gene Artemis are known to result in a form of severe combined immune deficiency (SCID). The incidence of this defect in the Athabascan-speaking Native American population is about 1/2000 live births. Here, we describe a case of an infant with SCID with the Artemis gene defect who presented with severe neutropenia despite a normal IgG level. Case: A 5-month-old Navajo female with a history of poor weight gain and frequent respiratory tract infections was admitted to the hospital due to hypoxemia and respiratory distress. Initial chest radiograph from the ED revealed bilateral infiltrates, and the child was placed on supplemental oxygen via nasal cannula. Respiratory culture was positive for respiratory syncytial virus (RSV) and rhinovirus. Initial lab work up revealed an abnormal complete blood count (CBC) with leukopenia, lymphopenia, and profound neutropenia (absolute lymphocyte count approximately 1000/mm 3 and an absolute neutrophil count of 300/mm 3 ). HIV screen was negative. IgG was preserved at 811 mg/dL, IgA was < 8 mg/dL, and IgM was 301 mg/dL. IgG subclasses revealed a total IgG at 799 mg/dL (IgG1 681mg/dL, IgG2 8 mg/dL, IgG3 6 mg/dL, and IgG4 undectable). The patient developed ecthyma gangrenosum of the nose secondary to infection at the site of nasal cannula with culture positive for pseudomonas species. The patient was put on anti-pseudomonal therapy with meropenum and ciprofloxacin. Immunology consult was obtained due to persistent severe neutropenia of unclear etiology. Recommendations were made for bronchoscopy, which revealed P. jirovici pneumonia and the patient was started on sulfamethoxazole/trimethoprim. T-cell receptor excision circles (TREC) assay revealed <74 TREC/10 6 cells suggestive of SCID. Due to her Navajo background, the patient was suspected of having a defect in the Artemis gene and was transferred to the University of California at San Francisco Medical Center for further management including a bone marrow transplantation. Conclusion: This is the first reported case of a child with a defect in the Artemis gene presenting with severe neutropenia and a normal IgG level. This unusual lab presentation led to a delay in immunology consultation and a definitive diagnosis. Although the patient's absolute lymphocyte count was also low, initial work up focused on the patient's neutropenia. D. Hirsch * , V. Bonagura, Great Neck, NY. Rationale: Hip replacement surgery is an increasingly common procedure in the United States, as more than 300,000 individuals undergo total hip replacement (THR) yearly. Common causes of joint replacement failure have historically included mechanical loosening, infection, fractures around the implant, and joint instability. Failure rates are as high as 6.1% for metal-on-metal THR and 3.3% for ceramic-on-ceramic articulations[i]. In the general population, 10-15% of individuals have metal hypersensitivity [ii] , which may represent an increasing cause of joint replacement failure. We describe the presentation, clinical course, and evaluation of a patient with recurrent infections after THR who was ultimately diagnosed with metal hypersensitivity. Methods: A 48-yearold man presented for an immunological evaluation because of recurrent infections after a THR. Three days after the operation, he developed a fever and underwent a washout and temporary insertion of a spacer. A few weeks later, he underwent re-implantation of hardware, but the wound did not heal properly so a spacer was inserted and he had another re-implantation of hardware 3 months later. One year after the original procedure, he developed an abscess and a fistula in the hip. He was treated with intravenous antibiotics and had another re-implantation of hardware. One year later, he again developed a joint infection which required removal of the hardware and implantation of a temporary spacer. Prior to the hip replacements, he had been very healthy with no significant infections. Results: After an immunological workup was unremarkable, patch testing to metals contained in the prosthetics elicited positive responses to nickel and chromium. Conclusions: It is important to include metal hypersensitivity in the differential diagnosis of individuals presenting with recurrent infections after joint replacement. Introduction: Shwachman-Diamond Syndrome (SDS) is a rare, autosomal recessive disorder characterized by pancreatic dysfunction, hematologic abnormalities, small stature, and recurrent infections. Dysgammaglobulinemia in SDS has been reported, but the use of Intravenous Immunoglobulin (IVIg) to reduce infection rate has not been studied. We present siblings with SDS treated with (IVIg) whose rate of hospitalization was dramatically reduced. Case Description: We present brothers, ages 15 and 17, diagnosed with SDS in early childhood, who have exocrine pancreatic dysfunction, small stature, and recurrent infections. Since diagnosis, they have required repeated hospitalizations for infections. Both had normal immunoglobulin levels, MBL function, total complement, neutrophil oxidative burst, and post-vaccination antibody levels. Since initiation of IVIg, both patients reported significant improvement in their quality of life with improved fatigue and less missed school days. Discussion: SDS is a rare congenital disorder with limited data on treatment options. Recurrent respiratory infections, beginning early in childhood, are frequently reported and often require hospitalization to rule out sequelae. While SDS may be associated with immunoglobulin deficiencies, a literature search did not reveal any publications documenting the use of IVIg to reduce infection rate in patients with SDS. In the two patients presented, the use of monthly IVIg has shown objective health benefits including decreased overall infection rate and decreased hospitalization rate; as well as subjective improvement in quality of life, improved fatigue, and decreased number of missed school days. This unique case study supports the use of monthly IVIg infusions in patients with SDS by providing objective data. It also supports the important need for the development of more scientifically supported clinical guidelines in the treatment of rare genetic conditions causing immune deficiencies. Introduction: 65-year-old female with linear scleroderma was evaluated for recurrent angioedema refractory to high dose steroids and antihistamines. She was initially found to have a mildly elevated serum tryptase, elevated serum histamine, and eosinophilia. Bone marrow analysis and cytogenetic studies were unremarkable. Serum abnormalities and angioedema resolved on high dose antihistamines and a modified mastocytosis diet. Methods: Immunological, genetic, and pathological evaluations of a patient, LL, with atypical angioedema were performed. Results: LL had a history of benign multinodular goiter and recent diagnosis of linear scleroderma. She presented with facial and both proximal and distal extremity angioedema that was poorly responsive to oral corticosteroids, antihistamines and plaquenil. There was no associated urticaria or pruritus and no identifiable exacerbating factors. C4 complement (44 mg/dL), C1 Esterase inhibitor (40 mg/dL), and C1q (8.3 mg/dL) were all normal. Quantitative immunoglobulins (IgG 1010 mg/dL, IgM 206 mg/dL, IgA 141 mg/dL) were normal. Serum protein electrophoresis was normal. Auto-antibodies (anti-DNA, anti-SCL70, anti-nuclear, anti-TPO, anti-TG) were negative. Over a period of 2 months, serum IgG decreased from 1010 mg/dL to 659 mg/dL and serum IgG2 from 206 mg/dL to 153 mg/dL. Serum histamine was elevated (8.7ng/mL) and tryptase was slightly elevated at 13 ng/mL. She acutely developed a marked hypereosinophilia, AEC 3200/mL up from a baseline of 400/mL, 2 months earlier. Parasitic workup was unremarkable. Bone marrow and cytogenetic evaluations for hypereosinophilic syndrome, mastocytosis, and myeloproliferative disorders was negative. She was started on hydroxyzine 200 mg daily and a low histamine diet with resolution of the angioedema, tryptase levels dropped to 6ng/ml and serum histamine to 2.2 ng/mL and eosinophils decreased to 400/ml. Conclusions: We report a case of atypical angioedema, mildly elevated tryptase levels and histaminemia which has been responsive to high dose antihistamines and modified histamine-restricted diet. Evaluations for mastocytosis and neoplasias have been unremarkable. T. Lee * , P. Busse, New York, NY. Acquired deficiency of C1 esterase inhibitor (ACID) is a rare disorder with recurrent episodes of self-limited angioedema that typically affects the skin or mucosal tissues of the upper respiratory and gastrointestinal tracts. An underlying defect of ACID may be an undiagnosed malignancy or autoimmune disease, therefore further investigation to rule out these disorders is critical. The management of a patient with ACID involves education about potential triggers (including surgery, trauma, medications), ready access to acute treatment, and in some patients consideration of a prophylactic treatment. We present an 82 year old male that developed multiple episodes of angioedema of his extremities and in an episode of airway swelling around age 75 years. A diagnosis of ACID was made by a decreased C4, C1-inh function, C1-inh protein level and a low C1Q. There was no evidence of an underlying malignancy. He was initially given C1 inhibitor concentrate (C1INHRP) for treatment of acute attacks and danazol for prophylaxis as his attacks were becoming more frequent. Initially his attack rate and severity decreased, but over the following years, he required nearly weekly rescue dosing of C1INHRP for acute attaks. His danazol was changed to aminocaproic acidwithout improvement. A high antibody titer to C1-inh was detected and the patient was also given icatibant for rescue medication. He was subsequently given a round of rituximab which significantly decreased his frequency of attacks and need for rescue therapy. After approximately 1 year, his angioedema symptoms increased in frequency and he was given another course of rituximab with improvement again, which waned over the following year. A third course of rituximab was administered. The patient has been monitor for lymphocyte numbers and specific antibody responses. In conclusion, treatment of ACID can be difficult and may require rituximab. Although additional courses of rituximab may be warranted, it should be done with careful monitoring of the patient's immune status. Background: Hereditary angioedema (HAE) is a rare disease, characterized by localized swelling, inflammation, and pain. It is the over production of bradykinin that leads to the angioedema. Icatibant (FIRAZYR®) is used to treat acute attacks of hereditary angioedema by inhibition of the bradykinin receptor. Icatibant is approved for use in type I and II, but is often used off label for type III. Icatibant is contraindicated in pregnancy and at this time no data are available on the safety of using icatibant in pregnancy. We report a case of type III HAE with frequent use of icatibant that suggests icatibant may be safe during pregnancy. Methods: The patient was first diagnosed with type III hereditary angioedema based on her history and normal compliment blood tests. C1inhibitor was given for prophylaxis. Icatibant was used frequently for attacks and continued to be used despite onset of pregnancy without the prescribers' knowledge. During the first two trimesters, icatibant was used multiple times a week. Results: Despite the use, two births resulted in normal fetuses without apparent teratogenic effects. One fetus was premature by 2 weeks and slightly below gestational weight; however, is physically normal and up to date on developmental skills. Conclusions: It appears that the use of icatibant early during pregnancy is safe; however, additional cases of accidental use during pregnancy are needed to confirm this suspicion. T. Ocampo * , T. Rans, San Antonio, TX. Eosinophilic granulomatosis with polyangitis (EGPA), formerly known as Churg-Strauss syndrome, is a rare multisystem, antineutrophil cytoplasmic antibody (p-ANCA) associated vasculitis characterized by asthma and hypereosinophilia. Its clinical manifestations are variable and often develop over years, making diagnosis challenging. We present a previously healthy 30 year old female marathon runner with abrupt onset rhinitis without polyps, productive cough of mucus plugs, intermittent dyspnea, and chest tightness while living in Missouri. Although diagnosed clinically with allergic rhinitis and asthma, antihistamines and inhaled corticosteroid (ICS) use provided minimal relief. Symptoms initially improved upon relocation to Texas, but occasional respiratory exacerbations occurred over the following year. Abruptly, during a bout of sinusitis, she endorsed fatigue, weight loss, burning leg pain, facial angioedema, and a blistering rash. Routine CBC demonstrated marked eosinophilia (AEC 16,100) prompting hospital admission. Initial investigation of primary (FIP1L1-PDGFRA) and secondary (medications, infectious, malignancy, rheumatologic) etiologies of eosinophilia were unrevealing. Rheumatoid factor (RF), antineutrophil antibody (ANA), and ANCA (PR3 Ab 0.5, MPO Ab 0) were negative. Sinus CT revealed extensive paranasal sinus disease. Chest CT revealed tree in bud and ground glass opacities, mucus plugging, and lymphadenopathy (normal chest CT the year prior). Serum IgE (3901 IU/ml) and CRP (1.4mg/dL) were elevated. Aspergillus precipitans were negative. Skin biopsy revealed eosinophil infiltration and findings consistent with vasculitis, which along with her constellation of symptoms led to the diagnosis of EGPA. Prednisone (75mg daily) was started with rapid resolution of peripheral eosinophilia and she has continued to clinically improve on a prolonged steroid taper. Retrospectively, this patient demonstrated the prodromal, eosinophilic, and vasculitic phases described in EGPA. However, her diagnosis was elusive to her multidisciplinary team until a rapid surge of symptoms prompted a skin biopsy. This case reminds the practicing Allergist of the importance of frequent reassessments and to maintain a high index of suspicion for alternative diagnoses in patients with emerging presentations. Introduction: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare, systemic vasculitis characterized by a prodromal phase of chronic rhinosinusitis, asthma, and eosinophilia. Treatment of asthma with CS (corticosteroids) may suppress clinical signs of EGPA. Further, agents such as omalizumab or leukotriene modifiers may enable weaning from CS, subsequently unmasking EGPA. We report a case of EGPA with onset following cessation of systemic CS and complicated by mesenteric vasculitis with bowel perforation. Case Presentation: A 56-year-old man with allergic rhinitis, chronic sinusitis, one-year history of steroid-dependent asthma, and eosinophilia (0.4-1.9K/uL) was hospitalized with severe epigastric abdominal pain, worsening dyspnea, and left upper extremity weakness and numbness that developed one week after abrupt discontinuation of oral prednisone. Asthma therapies included inhaled CS, long-acting bronchodilators, montelukast, systemic CS, and omalizumab. Extensive eosinophilia workup was previously negative. Physical examination on presentation was significant for bibasilar crackles, periumbilical tenderness, and decreased sensation to and weakness of the left hand. Data: WBC 26K/uL, absolute eosinophil count 15.2K/uL, CT chest: peribronchial ill-defined ground glass opacities and tree in bud nodules, BAL: 35% eosinophils, EGD: patchy erosions from distal duodenum to the middle jejunum, C-ANCA positive with a titer 1:256, Myeloperoxidase antibody elevated at 111 AU/mL. A diagnosis of EPGA was made and pulse dose methylprednisolone was initiated. Xolair and montelukast were discontinued. He was subsequently treated with Rituxumab 375mg/m 2 for four weeks. Six weeks following discharge, he developed severe abdominal pain and presented with an acute abdomen. Exploratory laparotomy revealed full thickness perforation of the proximal jejunum. Histopathological evaluation of the resected segment was consistent with eosinophilic vascultitis. Post-operatively, prednisone 25 mg and cyclophosphamide 100 mg daily have resulted in resolution of eosinophilia and improvement in symptoms. Conclusion: EGPA may be precipitated by CS withdrawal in patients with adult onset, steroid-dependent asthma. Consequently, clinicians should cautiously monitor for signs of EGPA when tapering CS in severe asthma patients, especially in those with eosinophilia. Background: Macrophage Activation Syndrome (MAS), a potentially fatal complication of sJIA (systemic juvenile idiopathic arthritis), is characterized by high persistent fevers, hepatosplenomegaly, lymphadenopathy, pancytopenia, hyperferritinemia, hypertriglyceridemia and liver dysfunction. Objective: To describe a case of sJIA and MAS requiring high dose anakinra (interleukin-1 receptor antagonist). Case Description: An 8-year-old female with sJIA improved with pulse methylprednisolone (30 mg/kg/day IV x 3 days), anakinra (3 mg/kg/day SQ), and tocilizumab (monoclonal interleukin-6 receptor antibody) infusion. Two weeks later, she developed unremitting fevers (105°F) and MAS was suspected. Results: She had hyperferritinemia, elevated d-dimers, transaminitis, and hypertriglyceridemia. Bone marrow biopsy revealed hemophagocytosis and anti-CD163 staining identifying activated histiocytes. She received high dose methylprednisolone (30 mg/kg/day IV x 6 days, then tapered over 7 months), cyclosporine, intravenous immunoglobulin, and anakinra (Table 1) for MAS. Anakinra was increased from 3 mg/kg/day to 12 mg/kg/day. Fever resolved in 4 days, laboratory markers improved and she was discharged home in 14 days. Medications were tapered over 12 months. She subsequently flared and was controlled on daily anakinra. She was then switched to monthly Canakinumab, and her disease continues to be in remission on Canakinumab. Discussion: We report a case of MAS occurring during the initial presentation of sJIA. Excess interleukin-1 can lead to the clinical and laboratory findings of sJIA and MAS, and the up regulation of interleukin-6. Uncontrolled production and activation of macrophages and T lymphocytes lead to MAS manifestations, including prolonged high fever, pancytopenia, coagulopathy, lymphadenopathy, hepatic insufficiency, and neurologic dysfunction. MAS can rapidly progress to multi-organ failure and death if not treated aggressively. Anakinra is a recombinant interleukin-1 receptor antagonist with a short halflife (~ 4-6 hours). The usual dosage of anakinra is 1-2mg/kg/day. Our patient responded to a 4-fold increase in standard dosing, without adverse events. Our experience suggests that high dose anakinra is effective and safe in refractory MAS secondary to sJIA. Background: The clinical evolution of anaphylaxis is highly variable, depending on the volume of antigen exposure and degree of sensitization amongst other factors. Such a reaction may even progress in a stepwise fashion with sequential allergen exposures. A phenomenon of "anaphylactic priming" can be conceived given that the magnitude of basophil, Langerhans and mast cell activation is quantitatively linked to the degree of IgE binding via FcεRI, FcεRII and Galactin 3 receptors. It thus follows that an intimate series of individually small allergen challenges may translate into a summation of effector cell activation and 'readiness' for histamine and vasoactive mediator release. We report a case of anaphylaxis that likely exemplifies this phenomenon. Case presentation: A healthy 35 year-old male with no prior atopic history was outdoors when he was bitten on the left leg by a fire ant. The reaction that ensued was limited to local swelling and irritation of the affected extremity. A few hours later, he ate lunch consisting of beef, sea bass and pesto. After 15 minutes, he developed facial and lip swelling, throat closure and dyspnea and heralded emergency medical services. His management in the field and in the emergency room entailed intramuscular epinephrine and intravenous diphenhydramine with relief of symptoms. Work-up in the allergy clinic found him to have an obstructive pattern on spirometry with reversibility. Skin prick testing demonstrated significant reactions to peanut, tree nut, dust mite, grass mix, Bermuda mix, weed mix, fire ant, horse, dog and cat. He was advised to avoid NSAIDs, sulphites, peanuts, tree nuts, fish and exposure to fire ant venom. He was prescribed oral cetirizine 10 mg with an epinephrine auto-injector and an albuterol inhaler to use as needed for anaphylaxis and/or bronchospasm. Conclusion: Anaphylaxis due to immediate hypersensitivity represents a state of clinically significant histamine and inflammatory mediator release from effector cells upon binding and cross-linking of IgE. The short chemical half-life argues that such mediators are released not only in remarkable quantity but in synchrony in this setting. Recent allergen exposure in the form of fire ant venom in this case may have recruited effector cells into a state of readiness, thereby orchestrating a more profound level of mediator release following the second allergen challenge. J. Regan * , W. Stevens, B. Stein, P. Avila, Chicago, IL. Good's Syndrome is a rare disorder associated with thymoma, hypogammaglobulinemia, low peripheral B cells, and abnormal cell-mediated immunity. Complications from this disease include increased incidence of infections and autoimmune diseases. Since the discovery of Good's Syndrome in 1955, the prevalence of this disease is estimated to be only approximately 5% of patients with thymomas. We present a case of a 62 year-old male with a history of Good's Syndrome status post thymectomy receiving intravenous immunoglobulin replacement who presented with mild dyspnea on exertion and right arm swelling. The patient was found to have a right upper extremity deep venous thrombosis (DVT). Interestingly, he was also incidentally found to be pancytopenic with a white blood cell count of 3, hemoglobin of 4.9, and platelets of 27. A bone marrow biopsy showed normocellular marrow with lack of red blood cell precursors and very rare megakaryocytic precursors concerning for developing aplastic anemia or pure red cell aplasia. Infectious workup including screening for human immunodeficiency virus, parvovirus, herpes virus, Epstein-Barr virus, and cytomegalovirus was unremarkable. Hypercoagulability workup for the DVT was negative including screening for Factor V Leiden, Prothrombin, Protein S, Protein C, Lupus Anticoagulant, and Antithrombin III dysfunction. The patient was started on prednisone 100mg daily and cyclosporine 250mg twice a day with improvement in anemia but very little response in his thrombocytopenia. Unfortunately, the patient re-presented 1 month later with hearing loss, elevated liver function studies, and acute renal failure attributed to cyclosporine toxicity and this medication was subsequently discontinued. As he continued to be thrombocytopenic, the patient was then started on N-plate weekly. The patient's condition continued to deteriorate with multiple hospitalizations for failure to thrive. His poor performance status precluded aggressive treatments including immunosuppression and the patient ultimately passed away. In summary, this is a very interesting case of a patient with Good's syndrome found to have pancytopenia. Introduction: Minocycline is a semisynthetic tetracycline derivative used in the treatment of acne vulgaris. It is associated with several adverse effects including drug-induced lupus erythematosus, drug reaction with eosinophilia and systemic symptoms, and serum sickness. There are few reported cases of anaphylaxis to minocycline. Objective: To describe a patient with acne vulgaris who developed minocycline-induced anaphylaxis. Case Description: A 16 year old female with acne vulgaris treated with minocycline presented with a 2 day history of diffuse, erythematous and pruritic rash, and fever (maximum temperature of 38.8°C). Symptoms began approximately one hour following minocycline ingestion and progressed to the development of arthralgias, emesis, edema of the hands and feet and throat swelling. Patient was tachycardic upon presentation in the emergency department. She received a 1 liter normal saline bolus, diphenhydramine and was admitted. Results: Patient had elevated c-reactive protein (12.6mg/dL) and sterile pyuria. Complete blood count, complete metabolic panel C1 Esterase inhibitor functional assay, antinuclear antibody (ab), anti-neutrophilic cytoplasmic ab, anti-double stranded DNA ab, complements C3 and C4, were within range. Blood and urine cultures were negative. Patient discontinued minocycline 2 days prior upon onset of symptoms when rash appeared. Patient was started on Solumedrol (2 mg/kg/day divided every 6 hours). On HD (hospital day) #2, she was switched to Prednisone 20 mg BID. Patient complained of "scratchy" throat, shortness of breath and flushing concerning for anaphylaxis. 0.3 mg of Epinephrine was administered intramuscularly. Patient's rash continued to improve and symptoms of edema, shortness of breath and fever resolved. Patient was discharged on HD#3 with Cetirizine, Ranitidine, 7-day steroid taper and EpiPen prescription. Discussion: Minocylcine-induced anaphylaxis has been discussed in a few case reports in the existing literature. Our patient developed symptoms 1 month after the initiation of minocycline. Patient's symptoms partially improved after treatment with antihistamines and steroids, but she required an intramuscular dose of epinephrine, and was continued on an oral steroid course. As minocycline continues to be one of the important treatments for acne vulgaris, clinicians must be vigilant for its potential adverse effects. Introduction: Drug Related Eosinophilia with Systemic Symptoms (DRESS) is a rare reaction to medications that occurs 2-6 weeks after drug exposure and typically presents with fever, rash, facial edema, lymphadenopathy, eosinophilia and transaminitis. Common medications are aromatic anti-convulsants, sulfonamides, and Dapsone with immunosuppression being a risk factor. Reactivation of herpes virus family is seen in 60-75% of the cases. The mainstay treatment is removal of the offending agent and high-dose steroids. Case Description: 17 year-old white male with history of sideroblastic anemia status post bone marrow transplant, immunosuppression, Posterior Reversible Encephalopathy Syndrome (PRES) since January, adrenal insufficiency and atopic diseases that presented to the hospital with a very pruritic, bright red rash, shaking chills and fevers. He was admitted for presumed adrenal insufficiency and graft-versushost disease leading to the rash. Rash was biopsied, he was started on hydrocortisone 15 mg TID and cefepime. Family remarked that the fevers and chills have been present since diagnosis of PRES that was being treated with levetiracetam, amlodipine, furosemide. Patient was also on Dapsone for prophylaxis. He continued to have fevers and absolute eosinophil count was noted to be 5,420 on steroids with elevated liver enzymes. Allergy was consulted, viral titers at this time were negative. All the mentioned drugs were discontinued and prednisone 100 mg daily was started with resolution of symptoms and laboratory abnormalities. A few months later, the patient had reactivation of EBV treated with Rituximab and developed erythroderma, mild transaminitis and later eosinophilia (1,240). Prednisone was increased back to 50 mg daily and symptoms resolved. Conclusion: DRESS is a rare reaction to drugs that can be difficult to diagnose and even more difficult to determine the culprit medication due to the delayed timing. There are common drugs associated with this disorder, such as dapsone and amlodipine, but multiple medications have been reported as individual cases including Levetiracetam and furosemide. Our patient was taking all four of these. Reactivation of herpes viruses is common and should be evaluated. Finally, immunosuppression is thought to be a risk factor and DRESS should be included in the differential in these patients presenting with rash, systemic symptoms and especially if eosinophilia is present. Rash biopsy site. S. Patel *1 , E. Nwaobasi-Iwuh 2 , A. Wolff 3 , 1. Summit, NJ; 2. Morristown, NJ; 3. Newark, NJ. Introduction: Anticonvulsant hypersensitivity syndrome (AHS) is a known potentially life-threatening complication of phenytoin, carbamazepine and phenobarbital therapy. Although its occurrence is rare, when AHS occurs, it often results in hospitalization and even death. Although the exact mechanism is unknown, it is thought to be an immunologic adverse drug event that is a Tcell-mediated, delayed hypersensitivity reaction. Case: An 8 year old male with a history of seizure disorder maintained on phenytoin for 10 days, presented to the ED with high fevers and a pruritic rash on his trunk, extremities and face. On exam, pertinent positives included a temperature of 102F and a generalized erythematous, maculopapular rash. Phenytoin was discontinued. His fevers and rash resolved within 48 hours without steroids. He was subsequently discharged only to return to the ED 24 hours later with worsening rash and a fever of 103F. On examination, he appeared ill with tachycardia and hypotension. His pharynx was erythematous with cervical lymphadenopathy and he had a generalized maculopapular, nonblanching rash. He was started on a dopamine drip, vancomycin and ceftriaxone for presumed sepsis. Results: His CBC revealed a WBC count of 14,000/ml, with 73% neutrophils, 11% lym-phocytes, 10% monocytes, 2% eosinophils. His LFTs were significant for an elevated AST of 80 U/L, and ALT of 100 U/L. Sepsis workup including blood cultures was negative. Skin biopsy was consistent with the diagnosis of drug hypersensitivity reaction. Discussion: The time from starting phenytoin and appearance of rash with other positive findings noted above were all consistent with AHS. The patient was treated with intravenous steroids and improved. AHS is unusual in that it occurs later than most other drug reactions; about 2-6 weeks after initiation of the offending agent. Frequently, the hallmark features of fever, rash, and lymphadenopathy are accompanied by multiorgan-system decompensation. Recognition of this syndrome, which may have variable presentations, is the key to prompt discontinuation of the drug and further management. This case demonstrates one such variable presentation and stresses the importance of recognizing a symptom free period prior to progression of the disease to the state of shock. Furthermore, it reinforces the importance of including AHS and DRESS in the differential diagnosis of sepsis. J. Giacinto Lawrence *1 , P. Ponda 2 , 1. Roslyn, NY; 2. Manhasset, NY. Introduction: FPIES is a disorder presenting as repeated bouts of emesis within 1-3 hours with or without subsequent diarrhea 5-8 hours after ingestion of an offending food. It is primarily a non-IgE-mediated hypersensitivity disorder commonly diagnosed during infancy. The pathophysiology although not yet fully defined, likely involves stimulation of antigen-specific T cells and a resulting proinflammatory cytokine cascade inducing increased permeability of intestinal mucosa. The gold standard for diagnosis is the oral food challenge (OFC). The most common food triggers are milk and soy though solid foods can also induce FPIES. We present a case of IgE-mediated soy allergy treated with years of avoidance that presented as FPIES upon re-introduction of soy. Methods: A 6 month old female with eczema who developed a facial rash after drinking soy formula was found to have positive serum and skin prick tests (SPT) to soy, milk and eggs (see Figure 1 ). She had an OFC for baked egg at approximately 3 years old and passed. One year after incorporating baked egg into the diet, the patient presented for a soy OFC. Results: The patient tolerated 5oz. (150mL) of liquid soy challenge without reaction and was subsequently discharged. She returned approximately 2 hours after initiation of the soy challenge due to emesis. The patient's vital signs remained stable; exam was unremarkable with the exception of pallor; no rash was present. She remained alert, responsive and talking with continued repetitive nausea and vomiting. She received IM Benadryl and Epinephrine and was subsequently transferred to the ER by ambulance for continued monitoring where she received fluids, steroids and an antiemetic. The patient recovered and continues to avoid soy. Conclusion: Although rare, cases of IgE-mediated allergy converting to FPIES have been reported. Although FPIES is thought to be a predominantly non-IgE-mediated reaction, the role of IgE remains unclear. A future role for other diagnostic measures, such as patch testing, to quantify the risks of food challenge has been suggested however more research is necessary before these tests can be recommended. Given these rare cases, there should be a higher awareness and vigilance when diagnosing or challenging a patient with prior IgE-mediated food allergy for possible non-IgE mediated reactions. Introduction: Chronic Eosinophilic Pneumonia (CEP) is an eosinophilic lung disease that is idiopathic and progressive. It is an uncommon disorder affecting females, usually of the fifth decade, who present with chronic respi-ratory and systemic symptoms. An atopic history is common but may not be present.The diagnosis is supported by eosinophilia greater than 1000cells/uL, biopsy or bronchoalveolar lavage, pulmonary function tests (PFT), imaging and the exclusion of other causes for eosinophilic lung disease. We present a case of an uncommon condition with an atypical presentation which illustrates the importance of having a differential diagnosis in cases that may seem ordinary. Case Presentation: A 64 year old female without a smoking history presented to clinic with a four month history of left posterior cervical painless lymphadenopathy. She denied any weight changes, fever, chills, hemoptysis, poor appetite, dyspnea or cough. Physical examination showed she was breathing on room air at 97% with clear lungs sounds bilaterally without egophony nor dullness to percussion. There were two lymph nodes which were smooth, nontender and mobile of 1cm. HRCT showed multiple mediastinal, and bilateral hilar lymphadenopathy measuring 1.3 to 2.2 cm with groundglass opacities in subpleural distribution with basilar predominance. No honeycombing, bronchiectasis, pleural effusion, consolidation, nodules nor masses were noted. She was referred to pulmonary clinic for workup for an inflammatory lung process. PFT showed a FEV1/FVC ratio of 82% and a diffusion capacity of 59%. Additional findings showed eosinophilia of 43.4%, with absolute eosinophil count of 5,000cells/uL. Hypersensitivity pneumonitis panel, ANA, ANCA, and anti-CCP were negative. IgE was elevated at 838IU/mL. She had a right lung wedge resection which showed patchy eosinophilic infiltrates and intra-alveolar macrophages indicative of chronic eosinophilic pneumonia. Discussion: The initial presentation of posterior cervical lymphadenopathy and lack of respiratory symptoms presented a diagnostic challenge. CEP shows peripheral consolidation with upper lobe predominance. Although the pattern of HRCT indicated an interstitial lung pneumonia such as usual or fibrotic subtype, biopsy confirmed it to be CEP. The combination of clinical history and supporting clinical studies will guide the clinician towards a diagnosis for appropriate management. J.G. Ghably *1 , A. Kapila 1 , G. Shaw 1 , T. Roy 1 , R. Byrd 1 , K. Guha 2 , A 59 year old male with a history of type 2 diabetes, peripheral vascular disease, chronic kidney disease, and multiple lacunar infarcts is hospitalized for pontine stroke and treated for klebsiella and enterococcus pneumonia and Clostridium difficile colitis. He was readmitted following month for oxygen desaturation and altered mental status eventually requiring intubation and mechanical ventilation. EEG and lumbar puncture revealed normal findings. Cultures of sputum and bronchial washings grew Klebsiella pneumonia, vancomycin resistant enterococcus, candida, HSV1 and he was placed on appropriate antibiotics. Stool studies were positive for C. difficile and he was continued on oral vancomycin. Patient developed transient eosinophilia that was initially attributed to underlying infection. Eventually, tracheotomy was performed and patient was placed on chronic mechanical ventilation. After completion of antibiotics, he continued to exhibit leukocytosis and eosinophilia, chest infiltrates remained stable, and his diarrhea had not resolved despite continued vancomycin treatment. Strongyloides titer tested normal so further infective workup was pursued revealing sinusitis, otitis media, and candida cystitis. Despite receiving appropriate treatments, leukocytosis and diarrhea persisted. Stool was tested for ova and parasites and results were positive for Strongyloides stercoralis. After receiving 3 doses of invermectin, his diarrhea and encephalopathy resolved and he was able to be weaned completely off ventilator. Immunodeficiency evaluation revealed elevated IgE levels, low IgM levels, and normal IgG and IgA levels. Hyperinfection with Strongyloides stercoralis is generally seen in immunocompromised hosts with prolonged steroid therapy, acquired immune deficiency, malignancy, or chronic illness. Since the disseminated infection has a high mortality rate, it is imperative to recognize the disease and offer early treatment. Ivermectin is the treatment of choice. This nematode primarily affects the gastrointestinal system and the lungs, but may rarely manifest in the central nervous system as mycotic aneurysm, intracranial hemorrhages, or vasculitis. Primary selective Ig M deficiency is a rare form of the immune disorder associated with severe life threatening infections and severe allergic reactions. Intravenous immunoglobulin is the only treatment modality available without clear benefits. Hypereosinophilic syndromes (HES) are characterized by persistent hypereosinophilia with eosinophilic infiltration and mediator release that cause damage to multiple organs. The peripheral blood hypereosinophilia is defined as eosinophils exceeding 1.5 x 10 9 /L on two separate occasions separated by at least one month. We describe a 53 year old female with a history of ovarian cancer, asthma, chronic rhinosinusitis with nasal polyps (CRSwNP) and eosinophilia who presented with chest pain of 4 days duration and was found to have an absolute eosinophil count (AEC) of 15. 4 x 10 9 /L. Her troponins and CKs were elevated. She denied initiation of any new medications. She was previously seen as an outpatient for eosinophilia, asthma and CRSwNP. At her most recent clinic visit 18 months ago, while undergoing chemotherapy with steroids for the ovarian cancer, her eosinophil count was 0. Her highest previous AEC was 8.85 x 10 9 /L. Previous workup for Churg Strauss and skin testing for Aspergillus fumigatus to evaluate for ABPA was negative. Workup included a peripheral smear showing absolute eosinophilia with normal morphology. ANA, cANCA, pANCA, tryptase and quantitative immunoglobulins were normal. She was HIV negative. Strongyloides serology as well as stool O &P were negative. An echocardiogram showed an ejection fraction (EF) of 59% and a small pericardial effusion. A cardiac MRI revealed a normal EF, subtle evidence of edema and diffusely elevated gadolinium ECV fractions thought consistent with myocarditis. FISH analysis was negative for the presence of a deletion of CHIC2, or translocations involving the FIP1L1 and PDGFRA genes. She was treated with methylprednisolone 60mg IV every 6 hours with decline of her AEC to 0.4 x 10 9 /L She was discharged home on prednisone 60 mg daily for 1 week with follow up with hematology for possible bone marrow biopsy and cardiology for a cardiac catheterization with biopsy. However, she was unable to follow up and presented 1 month later with chest pressure. She was found to have an AEC of 11.5 x 10 9 /L. She underwent an endomyocardial biopsy that showed eosinophilic myocarditis and was treated with high dose steroids with improvement. The most likely diagnosis is idiopathic HES. A bone marrow biopsy is pending to exclude other etiologies such as primary HES due to myeloproliferative disorder or systemic mastocytosis. P. Buddiga * , Fresno, CA. Introduction: Henna painting or temporary Henna tattooing traditionally known as "Mehndi" is a custom used for centuries in the eastern world for bridal parties or other celebratory occasions. The dyeing agent is Hennotannic acid and very rarely leads to an allergic reaction or an adverse skin hypersensitization. Nevertheless Henna tattoos or painting are usually mixed with paraphenylenediamine [PPD] to assist with drying as well as darkening the color hence beautifying the different patterns of Henna art. Methods: A 21 year old girl with history of allergic rhinitis on Allergen Immunotherapy presents for her weekly allergy shot and complains that 6 weeks ago she attended a bridal shower for one of her friends and along with another 6 girls in the same age range had Henna tattoos painted on their hands and feet. She noticed that a week later after placement it started to itch, burn and started to swell only at the site of the tattoo. She immediately applied itch cream and 1% Hydrocortisone with no resolution. She did not seek any professional medical care hoping it would resolve on its own. Henna tattoos usually dissipate by themselves on their own in 2 weeks. This case is clearly abnormal since the swelling and coloring is still very distinct at 6 weeks out from application. Upon a serendepity consultation in the Allergy shot room she was given high dose topical corticosteroid plus dimethicone and wrapping at the site with periodic ice compresses for comfort. No blisters or other adverse events occured and the lesion dissipated in 10 days with treatment. Conclusion: Henna is a potent contact dermatitis agent and must be skin tested or patch tested prior to application. It contains paraphenyldiamine that can be a significant trigger. Recommendations are to patch test this agent prior to use and if the Henna artwork does not dissipate in 2 weeks to see your local allergist or dermatologist for relief and resolution. ANNALS Introduction: Granulomatous disease is a well-documented complication of Common Variable Immunodeficiency, primarily seen in lung, but can also involve skin, liver, spleen, and the GI tract. Autoimmune and lymphoproliferative disorders are common associations. We present a case of unexplained granuloma involvement of skin and bone marrow in the setting of selective antibody deficiency and MGUS. Case Report: A 56 year-old man with history of vitiligo, shingles, and pneumonia presented with a 9-year history of chronic leg ulceration after falling on a boat anchor. Vascular work-up was negative. Lesions were recurrent despite wound care, antibiotics for secondary infections and hyperbaric oxygen, but were prednisone responsive. Exam showed numerous pustular ulcers on legs and was otherwise normal. Studies for HIV, hepatitis, cryogloblulins, ANA, RF, anti-SSA, anti-SSB, and ACEI were negative. MRI was negative for osteomyelitis. Multiple ulcer biopsies showed extensive granulomatous process with no vasculitis. Bacterial, fungal and mycobacterial cultures were negative. Immune evaluation showed low IgA and IgM and high IgG levels. SPEP/IFE was positive for IgG lambda monoclonal protein. Antibody titers to H. Influenzae, diphtheria, and tetanus were normal. Streptococcus titers were low despite repeated vaccination with Pneumococcal and Prevnar vaccines. Lymphocyte subsets showed low lymphocytes and NK cells and stimulation was low with PHA and tetanus. IL-1 and IFN-gamma responses were low. Complement levels, toll-like receptor, and IL-12 receptor assay were normal. CT chest did not show any ILD or lymphadenopathy, including mediastinal. Subsequent work-up for myeloma revealed normal bone scan and flow cytometry. However, bone marrow biopsy revealed numerous non-caseating granulomas and 15 % plasma cells with normal cytogenetic studies and negative cultures for bacteria, fungus, AFB and mycobacteria. Patient responded to treatment with prolonged steroids and IVIG for selective antibody deficiency. Trial off IVIG after six months resulted in increased sinus and wound infections and thus was resumed. Conclusion: Diminished cellular responses with IL-1 and IFN-gamma have been implicated in host defenses against mycobacterium; however numerous biopsy cultures in this case were unrevealing. Further studies are needed to elucidate the underlying etiology of granulomatous disease in immunodeficiency states. Introduction: Aspirin-exacerbated respiratory disease (AERD) usually join sinonasal polyposis and bronchial asthma exacerbated by ASA. Given the persistence of symptoms like rhinorrea is important to consider differential diag-noses. Cerebrospinal fluid (CSF) leak could be classified as traumatic and nontraumatic causes, it's considered idiopathic when no cause is identified, and benign conditions of elevated intracranial pressure are ruled out. The cardinal clinical presentation is persistent rinorraquia confirmed by cytology/cytochemical detection of CSF or ß2-transferrin as part of the diagnostic approach. Case Report: Female of 52 years old with 1 year of evolution with hyaline anterior rhinorrhea, persistent nasal pruritus, without identificable pattern of presentation, coughing and wheezing exacerbated with the intake of ASA; spirometry with bronchodilator reversibility of 14%, partial response to conventional therapy for persistent rhinorrhea, no trauma history, CT scan of nose and paranasal sinuses showed soft tissue density in entire left maxillary sinus, with concommitant occupation of anterior left ethmoid cells in intimate contact with cribriform plate. Cytochemical and cytological study confirms the presence of CSF. As intraoperative finding concommitant maxillary chronic rhinosinusitis were identified due to sinonasal polyposis, after endoscopic closure of CSF leak total control of symptoms were achieved. Discussion: AERD is a persistent inflammation condition of nasal mucosa, that has been linked to asthma exacerbations, sometimes sinonasal polyps are not evident clinically or endoscopically, its important to assess the specific anatomy of patients with suboptimal control sino-nasal symptoms. The non-traumatic CSF leak is one of the differential diagnoses that should be considered in patients with persistent rhinorrhea. Introduction: We report a case of a patient with 3 mechanistically different reactions to rituximab and a successful management approach. Methods: Case report Results: A 30 year old male with steroid-dependent oral pemphigus vulgaris developed subjective chest and throat tightness, hoarseness, and difficulty breathing 1 hour after his first rituximab infusion at an outside facility, which resolved within 15 minutes of stopping the infusion and treatment with intramuscular (IM) epinephrine. He was evaluated by us 2 years later for rituximab desensitization. Based on his symptoms, we considered the possibility of drug-induced vocal cord dysfunction, and he received his rituximab dose after premedication with H1/H2 antagonists and oral corticosteroids without any initial reactions. Eleven days later, he noted a pruritic, red facial and truncal rash along with severe joint pain which he self-treated by increasing his daily steroid dose. Serum sickness-like reaction was diagnosed, and he was recommended to receive high-dose corticosteroids post-infusion. He developed objective tongue, soft palate, lip, and facial swelling; pruritus; and throat tightness within 30 minutes of his next dose, which resolved over 1 hour with stopping the infusion and treatment with IM epinephrine, IV methylprednisolon, and diphenhydramine. Due to the immediacy of the visible angioedema, an IgE-mediated reaction was diagnosed. He underwent a 17 step rapid desensitization protocol to rituximab. The infusion was briefly halted at step 11 (32.5 cc/hr at 4mg/ml) after he experienced subjective chest tightness. It was restarted 30 minutes later at 20 cc/hr when the patient noted subjective lip edema and palmar pruritus without objective evidence of rash, angioedema, or wheezing. He was treated with IV diphenhydramine and lorazepam with improvement of symptoms. At a maintenance dose of 20 cc/hr, he developed 2 urticarial episodes that resolved with IM epinephrine, antihistamines, and montelukast. After desensitization, he completed 10 days of prednisone 60 mg with no recurrence of the serum sickness-like reaction. Conclusions: Allergists should be familiar with the various adverse reactions to rituximab. This case illustrates a patient with multiple reactions (including serum sickness) who successfully received rituximab via desensitization and post-infusion corticosteroid therapy. L. Rampur, A. Rubinstein, Z. Ren * , Bronx, NY. Introduction: Recurrent abdominal pain is frequent in patients with hereditary and acquired angioedema. We present a case of recurrent abdominal pain presumed to have abdominal angioedema, found to have eosinophilic segmental serosal and muscular enteritis. Methods: Case description Results: 31 year female was referred for episodic abdominal pain since 2007, recurring every 3 to 6 months lasting several days. She was presumed to have abdominal visceral angioedema in 2009 treated with systemic steroids with relief of symptoms within 2-3 days. MRI during several episodes was consistent with localized Jejunal wall edema in the same 5-6" segment. Lab work at baseline and during attacks revealed normal C1 esterase inhibitor function of 102%, normal C4 (20mg/dl), C3 (97mg/dl), Tryptase (5.9 ug/L), autoimmune antibody panel, Celiac panel, alpha 1 anti-trypsin and quantitative immunoglobulin assay including ESR, electrolytes, liver and renal function and stool examination. But C1q level was low (< 1.2 ugeq/ml). Absolute Eosinophil count was1400k/ul (15%) when she was asymptomatic and as high as 3500k/ul (33%) during an episode. IL-5 levels were normal. FISH analysis did not detect translocations or deletions of the chromosome 4q12 resulting fusion of FIP1L1 and PDGFRA genes and rearrangement of PDGFRB and FGFR1. Peripheral blood did not show a mutation for JAK2 V617F myeloproliferative disorder and and molecular analysis showed normal alleles detected RT-PCR for BCR-ABL P210. Multiple upper endoscopies and colonoscopies including capsule endoscopy have been unremarkable. In a recent admission a MRI showed further spread of the jejunal thickening to around 10". A laparoscopic jejunal biopsy of the edematous region showed dense serosal and muscularis propria eosinophil infiltrates suggestive of eosinophilic enteritis, not involving the jejunal mucosa. Conclusion: This patient's presentation of recurrent abdominal pain with localized jejunal wall edema was suggestive of abdominal angioedema attacks. Work up for hereditary or autoimmune angioedema was negative. The only clue for the diagnosis was the fluctuating peripheral eosinophilia albeit with absence of increased eosinophil infiltrates in mucosal jejunal biopsies. Hyper eosinophilic syndrome was excluded by the absence of the relevant mutations in FIP1L1/PDGRA. Diagnosis was obtained by serosal and muscularis biopsy of the edematous jejunal segment. Introduction: A deficiency in C1 esterase inhibitor (C1-INH) can be caused by hereditary angioedema (HAE) or acquired angioedema (AAE). HAE occurs from a gene mutation that leads to either lack of C1-INH or production of nonfunctional C1-INH. AAE results from consumption or inactivation of C1-INH, usually in the setting of lymphoproliferative disorders. Several cases of AAE have been reported in conjunction with monoclonal gammopathy of undetermined significance (MGUS) and non-Hodgkin's lymphoma. We report a rare case of a patient, who presented with AAE, leading to a diagnosis of multiple myeloma (MM). Case: A 70-year-old male with asthma and chronic idiopathic urticaria presented to the ED with tongue and right anterior neck swelling without urticaria. Because his uvula could not be visualized on presentation, he was intubated. The patient's admission laboratory results were indicative of anemia, acute kidney injury and hypercalcemia. They also showed an increased globulin gap, an elevated serum IgG level, high serum free lambda light chains and a low C4. The patient's presentation was concerning for AAE due to an underlying malignancy. His angioedema did not improve approximately 12 hours after administration of intravenous steroids in the ED. The patient received 1 dose of C1-INH concentrate and within a few hours his angioedema rapidly regressed. C1-INH and C1-INH functional levels were normal, but the C1q level was found to be low, suggestive of AAE. A bone marrow biopsy revealed 35% plasma cells, consistent with a diagnosis of MM. The patient's angioedema resolved after administration of C1-INH concentrate and initiation of treatment for his MM. Discussion: The most common malignancies known to cause AAE are indolent lymphomas. However, we report a unique case of MM manifesting as AAE. The patient in our case suffered from AAE likely secondary to autoantibodies against C1-INH that are produced by aberrant plasma cells. Mechanistically, it is believed that autoantibodies bind to and destabilize the C1-INH complex, leading to proteolytic cleavage and inactivation of C1-INH and downstream generation of bradykinin. Conclusion: To our knowledge, this is the first reported case of AAE due to MM. This case highlights the principle that the management of AAE requires treatment of its underlying cause, which may be an autoimmune disorder or a malignancy. Introduction: Human enteroviral infections are the most common cause of meningoencephalitis in the United States. Enteroviral infection is usually a selflimited disease. However, in an immunocompromised patient, infections can be fatal. We present a case of an adult female patient with a history of Evan's syndrome treated with prior splenectomy and rituximab who subsequently developed panhypogammaglobulinemia after which, despite receiving antibody replacement, she developed a progressive and ultimately fatal meningoencephalitis due to human Coxsackie B3 virus. Case Description: A 28-year-old white female presented to the University of Virginia with a 4-month history of progressive encephalopathy. She had a history of Evan's Syndrome (autoimmune hemolytic anemia and idiopathic thrombocytopenia purpura) for which she had received treatment with rituximab and splenectomy 5 years prior to presentation. 5 months previously, she was hospitalized for Influenza A with concurrent facial Pseudomonas cellulitis and bacteremia. During that admission, she was found to have severe panhypogammaglobulinemia with 0% circulating CD19+ B cells and intravenous immunoglobulin (IVIG) was started. Her exam on admission was also consistent with aseptic meningitis. She was subsequently readmitted with worsening encephalopathy and repeat lumbar puncture and MRI were negative for an infectious process. A stereotactic brain biopsy was obtained, which was also negative for infection. Despite empiric treatment with broad-spectrum antibiotics, she continued to decline neurocognitively and hospice care was pursued. The patient expired and subsequent post-mortem brain biopsy identified human Coxsackie virus B3, on the basis of positive viral protein (VP)2, VP4 and VP 5` untranslated region (UTR) by PCR. Conclusion: Previous treatment of this patient with rituximab for Evan's syndrome was associated with persistent profound B cell lymphopenia and secondary hypogammaglobulinemia. Given the observation that some patients treated with rituximab will develop persistent lymphopenia and hypogammaglobulinemia, and the increasing use of rituximab in the treatment of malignancies and autoimmune disorders, physicians must have increased awareness of the risk of secondary B cell and antibody deficiency and susceptibility to enteroviral meningeal infections in this population. J. McCracken *1 , C. Schlegel 2 , S. Sur 2 , 1. League City, TX; 2. Galveston, TX. Introduction: Kounis syndrome is a rare condition when allergy or hypersensitivity can lead to cardiovascular symptoms. Symptoms are caused by the release of inflammatory mediators from mast cells which can have cardiovascular effects including coronary spasm. Methods: We report the case of a 61 year old male with a history of allergic rhinitis, asthma, monoclonal gammopathy of undetermined significance (MGUS) and eosinophilic esophagitis who was referred to us for evaluation of urticaria, chest pain and shortness of breath (SOB). His symptoms have been occurring over the past 2.5 years and rash typically precedes the cardiac symptoms. Six months prior to his first visit in our clinic, he had an acute myocardial infarction requiring stent placement. Physical exam was normal. Laboratory work-up was significant for elevated eosinophils, elevated IgE, and intermittently elevated tryptase with these episodes. Skin prick testing was positive for trees, weeds, grass, dust mite, cockroach, and cat. Bone marrow biopsy was c-kit negative. He was started on fexofenadine 180 mg in the morning, cetirizine 10 mg in the evening, and ketotifen 1 mg twice a day. Two weeks later, he again had chest and jaw pain associated with development of a rash on his palms that required hospitalization. Angiography showed patent stent. His medications were increased to fexofenadine 180 mg 2 tabs in AM, cetirizine 10 mg 2 tabs in PM and ketotifen 2 mg twice a day. He was unable to tolerate the increased doses of fexofenadine and cetirizine so he resumed his initial dosing of those medications. He now reports symptoms improved since last follow-up. Discussion: We believe his presentation of urticaria associated with severe chest pain and SOB is suggestive of Kounis syndrome. Current literature focuses more on the acute treatment of the allergic and cardiac symptoms, and less on the prevention of symptoms. In this patient, ketotifen, a mast cell stabilizer, and non-sedating antihistamines were utilized to control symptoms. Conclusion: We present a case of possible Kounis syndrome in a patient who is successfully being treated with non-sedating antihistamines and ketotifen. This reports to the allergists that this combination therapy can be a useful management option when treating patients with Kounis syndrome. Introduction: Perioperative anaphylaxis is a life threatening IgE or non-IgE mediated condition presenting with symptoms including urticaria, angioedema, bronchospasm, arrhythmias, and hypotension. 1 Its prevalence has been reported from 1:3,500 to 1:20,000 with a mortality rate of 3 to 9%. The most common culprits include antibiotics, neuromuscular blocking agents, latex, hypnotics, opioids, colloids, dyes, and nonsteroidal anti-inflammatory medications. Case Description: The patient was a 24 year old female with a history of asthma, rhinitis, egg allergy, eczema, and prior tonsillectomy who presented after two episodes of anaphylaxis while undergoing oral jaw surgery. During the first surgery she developed hypotension, wheezing, and hypoxia leading to cardiopulmonary arrest. She was treated with epinephrine, fluids, and corticosteroids. Skin prick testing was found positive to vecuronium, succinylcholine, rocuronium, cisatracurium, penicillin, sulfa, ciprofloxacin, and cefazolin. These agents were avoided during the next operation, but systemic symptoms recurred. Further evaluation showed a normal FEV1 with 14% reversibility post bronchodilator and an exhaled nitric oxide (eNO) of 52ppb indicative of airway inflammation. Urine tryptase and thyroid stimulating hormone (TSH) were normal. She was started on an inhaled corticosteroid. Twenty four hours prior to her third surgery she was treated with corticosteroids and antihistamines, and neuromuscular blocking agents and beta blockers were avoided. She tolerated her subsequent surgeries without complication. Discussion: This case illustrates the serious nature of perioperative anaphylaxis. Despite avoidance of the sensitized agents, anaphylaxis reoccurred. This led to further exploration discovering poorly controlled asthma which is a known risk factor for anaphylaxis. 2 P185 LYMPHOPENIA AND AGAMMAGLOBULINEMIA OF UNCLEAR ETIOLOGY. L. Bornstein * , R. Herzog, New York, NY. Introduction: Early diagnosis of severe combined immune deficiency has led to timely treatment and improved prognosis for patients. We present a 17 month old male with lymphopenia and agammaglobulinemia of unclear etiology identified by TREC assay on newborn screen. Case Report: The patient was a full term newborn with low T-cell receptor excision circle (TREC) levels. The patient was well until 53 days of age, when he developed fever to 38.5C and upper respiratory infection symptoms. Labs revealed low T and B cell counts and agammaglobulinemia, and he was treated with antibiotics and intravenous gamma globulin (IGIV). Cultures and viral PCR were negative. Thereafter, the patient developed recurrent otitis media and viral infections due to RSV, coronavirus, rhinovirus, enterovirus, and metapneumovirus. In addition to IGIV, the patient has been given prophylaxis with palivizumab, acyclovir, pentamidine (due to intermittent neutropenia on TMP-SMX), and filgrastim as needed. The patient has been developing normally for his age. Results: Newborn TREC was 132, 132, 164 (nl>200/ml), with repeat of 0, 0, 0, 31, 33/ml. Naïve CD45RA+ CD4 count was normal. Absolute lymphocyte count (ALC) was 900/ml (1,000-4,800), CD4+CD3+ 468/mm, 48% (2,780-3,908, 50-57%), CD8+CD3+ 127/mm, 13% (351-2,479, 8-31%), CD19+ 63/mm, 7% (432-3,345, 11-45%), normal NK function, IgG 522, IgA: <7, IgM 9 (20-87 mg/dl). T cell proliferative response to phytohemagglutinin (PHA), Concanavalin (con A), OKT3, and mixed lymphocyte culture was normal. Responses to Candida and Tetanus were low normal. There was no evidence of maternal engraftment or clonal T or B cells. Immunization with DTaP did not result in increased titers. Anti-neutrophil antibodies were negative. Artemis, RAG1, RAG2, adenosine deaminase (ADA), purine nucleoside phosphorylase (PNP), DNA ligase 4, TAC1, BTK, and 22q11 deletion studies were negative and telomeres were not consistent with dyskeratosis congenita. Thymus was identified on ultrasound. Conclusion: Early detection of T cell lymphopenia has led to early treatment and improved prognosis of severe combined immune deficiency. We present a case of T and B lymphopenia and IgM and IgA deficiency of unclear etiology to date. Further studies including whole exome sequencing may reveal the etiology. A 24 year old male presented to the allergy clinic with a four month history of persistent clear left nasal drainage. Patient denied any history of facial trauma, facial surgery, nasal polyps, or nasal deviation. Beyond the rhinorrhea, the review of systems was otherwise negative. Prior to presenting to allergy clinic, the patient was seen by his primary care physician and was on daily cetirizine, montelukast, and fluticasone nasal spray without any symptom improvement. Given the persistent unilateral rhinorrhea without history of trauma or surgery, further diagnostic workup was pursued. Computed tomography sinus imaging revealed a 6.5 x 5.6 x 4.4 cm infiltrative, destructive mass centered in the sella turcica with intracranial nasopharyngeal extension which was subsequently confirmed by MRI. Laboratory results were remarkable for a positive beta-2 transferrin and an elevated prolactin (532, normal range 3-13 ng/mL). An endoscopic left nasopharyngeal biopsy was performed with pathology consistent with an atypical pituitary adenoma. The staining profile of the adenoma was of a prolactinoma. The patient subsequently underwent neurosurgery for repair of the CSF leak followed by treatment with cabergoline. Conclusion:Nasal symptoms such as rhinorrhea are commonly encountered in both the allergy and primary care clinics. Frequently, these symptoms are related to inflammatory conditions such as sinusitis, rhinitis, and nasal polyps. However, rarely they may be related to malignant lesions. Red flags of a unilateral distribution along with a refractory response to standard medical treatment should prompt the physician to examine for other differentials including sinus masses. MRI demonstrating a infiltrating mass in the sella turcica with biopsy and pathology consistent with a prolactinoma. Rituximab is a B cell depleting anti-CD20 monoclonal antibody often used in the setting of malignancy or autoimmune conditions. Transient B cell depletion is often seen but immunoglobulin levels are not often affected 1 . However, there is growing literature documenting hypogammaglobulinemia requiring intravenous immunoglobulin following rituximab therapy 1,2 . In some cases, the presentation of hypogammaglobulinemia was years after completion of rituximab therapy 3 . We present the case of a 71 year old female with a history microvascular polyangiitis treated with rituximab, cyclophosphamide and prednisone who was diagnosed hypogammaglobulinemia, specifically IgG deficiency, two years after completing therapy. The patient had a history of recurrent pneumonias preceding the immune modulating regiment, however all episodes required only outpatient antibiotic therapy. After completion of treatment the patient required frequent inpatient admissions for pulmonary infections, including three to the intensive care unit. Subsequent evaluation revealed an IgG of 412 mg/dL, IgM of 31mg/dL and IgA level of 129 mg/dL. She had an appropriate antibody response to pneumococcal polysaccharide vaccine with 11 of 23 titers positive. B cells were present in normal numbers. Thus, a diagnosis of IgG deficiency was made 4,5 . She was started in intravenous immunoglobulin therapy and has been well since initiation of treatment. This patient adds to the cases in the literature of hypogammaglobulinemia requiring immunoglobulin therapy after rituximab treatment. In our patient the immunoglobulin levels prior to initiation of therapy are not available so it is not known if treatment accelerated an already existing condition or represents a truly new illness following therapy. There are case reports of clinically asymptomatic patients with reduced immunoglobulin prior to rituximab treatment that experienced clinical deterioration after therapy 1 . It is possible a similar scenario occurred in our patient given the history of frequent, though milder, infections prior to therapy. Retrospective studies have shown that only a small minority of patients develop hypogammaglobulinemia following rituximab therapy indicating there is likely a specific subset of patients at risk 6 . More research is needed to determine if this association is due to an aggravation of an existing condition and the utility of screening. Introduction: The development of Factor IX (FIXi) inhibitor (antibodies) in severe Hemophilia B (FIX deficiency) patients is a rare phenomenon (1%-6% incidence), increasing morbidity and mortality. Treatment of these patients can be complicated by the association of FIX inhibitor (FIXi) with anaphylaxis to FIX replacement therapy. More recently, the use of rituximab to eradicate inhibitors has been reported. Methods: We describe two Hemophilia B patients (Pt#1-8 year old and Pt#2-4 year old males) who developed anaphylaxis to recombinant FIX replacement product, BeneFIX (RFIX), in the setting of an inhibitor. Results: After 9 months of RFIX therapy for the treatment and prevention of bleeding episodes, Pt#1 developed anaphylaxis shortly after RFIX infusion. FIXi titer was 4.3 BU. Pt#2 was receiving RFIX therapy that was discontinued due to detection of a FIXi of 0.6 BU on routine screening. After his inhibitor titer became undetectable, he was re-challenged with RFIX. He developed an anaphylactic reaction after 5 weeks of RFIX therapy. FIXi was 2.4 BU. Skin testing to RFIX and plasma derived FIX, Mononine (PFIX), was performed for both patients. Pt#1's skin test was negative to RFIX but positive to PFIX. Pt#2's skin test was negative to both products. The patients were treated with rituximab 375mg/m2/dose weekly x 4 and successfully completed desensitization to RFIX. Pt#1's FIXi decreased to 0, and he has been tolerating RFIX with no resurgence of his inhibitor for 9 months thus far. Pt#2's FIXi and Bcells dropped from 6.3 BU to 0 and 603 cells/uL to 0 respectively. After 6 months of tolerance, Pt#2 developed diffuse urticaria and dyspnea within minutes of completing his RFIX infusion. FIXi was 0.8 BU, and B-cells were 270 cells/uL. Repeat skin testing was minimally positive to RFIX and negative to PFIX. Subsequently, patient failed graded challenge to PFIX (generalized hives). Conclusion: These cases highlight the importance of recognizing that an inhibitor can play a role in anaphylaxis to biologic agents. Skin testing to FIX preparations is not necessarily predictive of systemic reaction. Rituximab has been used to eradicate the inhibitor in some patients. As B-cells recover, the inhibitor may reappear in selected patients, and maintenance rituximab may need to be considered. J.T. Abraham * , J. Fernandez, Cleveland, OH. Background: Autoimmune Progesterone Dermatitis is an IgE mediated reaction to endogenous or exogenous progesterone. Clinical presentations are described as varied, with cutaneous lesions mostly on abdomen and lower extremities. The rash occurs cyclically, typically during the luteal phase of the menstrual cycle when progesterone levels are high. Methods: A 28 year-old healthy Caucasian female presents to Allergy and Immunology clinic with intermittent lower extremity and abdominal rash since May 2013. The erythematous maculopapular rash occurred every 4-6 weeks initially, then increased in frequency. It normally lasted three days and self-resolved. The rash progressively became associated with myalgias and malaise. She had been using a NuvaRing for contraception during this time. Results: The patient was initially evaluated by rheumatology with concern for the rash being consistent with vasculitis. Skin biopsy was performed, demonstrating perivascular dermatitis with eosinophils and neutrophils as well as extravasation. Laboratory evaluation was remarkable for elevated IgG levels (2960 mg/dL), a speckled pattern ANA (1:1280), positive SSA (122 units) and SSB (75 units) antibodies. She was sent to Allergy and Immunology for evaluation due to the cyclical nature of her rash and concurrent use of hormonal contraception. Epicutaneous and intradermal skin testing was performed with progesterone (50mg/ml) as well as positive (histamine) and negative (normal saline) controls. Epicutaneous testing with 1:1 concentration was negative. Intradermal injection of 1:1000 and 1:100 dilutions showed positive wheal/flare reactions of 5mm x 12mm and 8mm x 15mm, respectively. Intradermal injections were also placed in three healthy subjects to ensure the dilutions used were not irritant doses. The healthy controls showed minimal to no reaction to these dilutions. Conclusions: Autoimmune Progesterone Dermatitis is a rare condition, however it should be considered in women of child bearing age who present with cutaneous lesions in a cyclic pattern. Introduction: Methimazole is a common reagent used to treat hyperthyroidism. Its side effects such as rash, vasculitis, have been widely reported but rarely has an IgE mediated reaction been reported in a child and no desensitization protocol exists for this medication. We present a case of IgE mediated allergic reaction after administration of methimazole on two occasions along with a successful desensitization to the medication with subsequent treatment of hyperthyroidism. Case Description: A 7 year old Hispanic female with a history of intermittent asthma and hyperthyroidism treated with methimazole and developped a generalized itchy rash and hives with myalgias and arthralgias. Her symptoms resolved within 1 day after stopping the medication. She was then switched to propylthiouracil (PTU) and had no issues. Another trial of methimazole lead to the same symptoms and the patient was again switched to PTU which result in elevated liver enzyme within a few months. The patient presented to our allergy clinic for evaluation. Skin prick testing showed a positive reaction to methimazole at 100 mcg/ml concentration. Intradermal testing showed a 6 mm wheal to the 1 mcg/ml concentration. The starting dose of desensitization was calculated to be 100 fold less than that which yielded a 5-8 mm wheal by skin testing. Patient was monitored in our clinic for possible local and systemic reaction. Desensitization was started at a concentration of 10 ng/ml in a volume of 0.3 ml (dose of 3 ng) with no reaction. The dose was increased half log every 15 minuntes until a cumulative dose of 29.5 mg was reached. Our patient had no reactions during the desensitization and was started on a dose of 5 mg daily and increased to therapeutic dose for her hyperthyroidism. Conclusion: Drug desensitization protocols exist for a few medications mainly beta lactam antibiotics, vancomycin, aspirin, NSAIDS and some chemotherapy reagents and monoclonal antibodies. To our knowledge, no methimazole desensitization case has been reported. Our report provides a useful tool in treating hyperthyroism of patients with allergic reaction to methimazole who are not tolerating PTU. A 15-year-old male with a history of hereditary angioedema (HAE) type 1 presented to the local children's hospital emergency department (ED) with a chief complaint of 4 days of sore throat and abdominal pain. He had 7 prior admissions for HAE attacks, with 2 in the past year. His typical attacks included angioedema of his feet and face, and occasionally abdominal pain. He was always treated with C1 esterase inhibitor. Preventative therapy had been discussed at his prior allergy clinic visits, however he had been lost to follow-up prior to this presentation. On his current presentation, the patient noted that his abdominal pain was similar to prior flares, but denied any emesis, dyspnea, or angioedema. He had multiple sick contacts at home with similar symptoms. On exam, the patient had stable vital signs. He appeared to be in no acute distress. On HEENT examination he had clear tympanic membranes, PERRLA, clear oropharynx with 2+ tonsils with mild erythema and without palatal, tongue, or uvula edema, moist mucous membranes, and supple neck. His lungs were clear to auscultation, and heart had regular rate and rhythm without any murmurs. His abdominal examination was positive for mild tenderness to palpation diffusely without rebound or guarding. His skin exam was unre-markable and there was no swelling or edema noted. A rapid strep test was performed. Approximately 30 minutes passed and on reexamination the uvula was noted to be edematous. The patient then began to have progressive palatal edema that involved the entire posterior oropharynx. The patient had no respiratory distress and continued to have stable vital signs, however the decision was made to electively intubate him to protect his airway. C1 esterase inhibitor therapy was initiated at this time. On direct laryngoscopy, the patient's palate was noted to be edematous, however the glottis, trachea and larynx were all normal. The patient remained intubated for less than 24 hours, was successfully extubated and discharged to home within 72 hours. Our case of an HAE attack with isolated palatal edema after the rapid strep test was performed raised the unique question: did the rapid strep test incite enough trauma to the posterior oropharynx to provoke an attack of HAE? Our case suggests that the patient was at the beginning of an attack and the trauma of the rapid strep test may have caused him to have isolated angioedema to the posterior oropharynx. 41-year-old female with past medical history of Gastroesophageal Reflux (GERD) well-controlled on famotidine and obesity status post lap-band surgery three years prior presented with a one year history of cough. Her cough was intermittently productive of yellow (morning) to clear sputum (later in the day) alternating with dry cough that was worse with exertion and associated with wheezing and dyspnea. She had frequent nocturnal awakenings. She also had perennial clear rhinorrhea with postnasal drip, nasal and ocular pruritus and sneezing. Social history was notable for cat and dog at home and tobacco use. Physical exam was unremarkable except for obesity, clear rhinorrhea, postnasal drainage and non-productive cough. Percutaneous skin testing was positive for cat and dog. Pulmonary function tests (PFTs), chest x-ray, and sinus CT were normal. She was diagnosed with perennial allergic rhinitis, cough and GERD. Initial treatment included environmental controls, a nasal steroid and albuterol for rescue. She continued to have nocturnal rhinitis and a wet cough. Pulmonary function tests progressively declined and demonstrated a positive bronchodilator response. Management over the next few years included trials of immunotherapy for cat and dog, high dose proton pump inhibitor, antibiotics, montelukast, steroids (nasal, inhaled and oral) and omalizumab. Cough with frequent nocturnal awakenings continued to be her main complaint. GERD was repeatedly assessed and reported to be well-controlled by the patient. Approximately four years after initial presentation, chest CT and esophagogastroduodenoscopy (EGD) were performed due to worsening of symptoms and oral steroid dependence. The CT showed a fluid distended thoracic esophagus, and EGD revealed food in her esophagus despite being NPO the night prior to the procedure. She recalled two episodes over the past several months of coughing up food that she had eaten 24-48 hours earlier. It was subsequently realized that her lap-band was inflated one to two years prior to the initial onset of her symptoms. Her lap-band was deflated with dramatic improvement in symptoms and PFTs. She was able to discontinue oral steroids and maintenance asthma medications. There is one other report of chronic cough and nocturnal rhinorrhea following lap-band surgery. Chronic cough is a complication of lap-band that may be under-recognized outside the general surgery community. Introduction: Although NTM infection is known to exist in patients with chronic lung diseases, it is seldom considered in the evaluation of severe or difficult-to-control asthma. Case Report: A 66 year old woman was referred to the allergy clinic with difficult-to-control asthma. She had recurrent daytime productive cough with yellow sputum and frequent nocturnal awakening. She described repeated use of short acting β agonist despite compliance with combination (high dose) inhaled corticosteroids (ICS)/long acting β agonist and leukotriene receptor antagonist. Pulmonary function tests were consistent with severe obstructive ventilatory limitation. A High resolution chest CT scan showed multiple bilateral pulmonary nodules. She had two positive sputum cultures for Mycobacterium avium-intracellularae and was started on ethambutol, rifabutin and azithromycin. She has had marked improvement in symptoms and decrease in albuterol use since starting treatment. Discussion: A recent study showed that the estimated prevalence of NTM infections among asthmatic patients was 1.7% with an average interval of 2 years from symptom change to diagnosis. Patients commonly present with symptoms of worsening lung disease including progressive dyspnea, productive cough and frequent asthma exacerbations. Imaging studies may reveal bronchiectasis, nodules < 5 mm or fibrosis. American Thoracic Society (ATS)/ Infectious Diseases Society of America (IDSA) guidelines require at least two positive expectorated sputum cultures for diagnosis of NTM infection. Recent studies have indicated that older patients with severe airflow limitation receiving long term high dose ICS are at a higher risk of acquiring NTM infection. The current recommended treatment for pulmonary disease comprises a triple drug regimen with azithromycin, ethambutol and rifabutin for duration of at least 12 months after conversion of sputum to negative. It is imperative to consider NTM infection in the differential of difficult-to-control asthma that has been unresponsive to conventional therapy. S. Waqar *1 , S. Farzan 2 , 1. Dix Hills, NY; 2. Great Neck, NY. Introduction: Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) is a rare, but potentially life-threatening syndrome, which typically presents two to eight weeks after drug exposure. It is characterized by multiple system involvement, which may include skin eruption, hematologic abnormalities, lymphadenopathy, and affect internal organs such as the liver, kidneys, and lungs. Patients usually face several relapses despite discontinuation of the drug. Here we describe the presentation, diagnosis, and clinical course of a patient with early-onset DRESS. Methods: A 15-year-old male, with past medical history of hypertension presents with recurrent pruritic rash and shortness of breath two weeks after treatment of a MRSA skin infection with trimethoprim-sulfamethoxazole (TMP-SMX). He reports initial development of a rash on day 4 of TMP-SMX. The rash was a full-body pruritic erythematous papular rash sparing the mucus membranes and most prominent on the face. TMP-SMX was discontinued. He was treated with an oral prednisone taper, as well as intravenous fluids for acute kidney injury with a creatinine of 1.5. The rash resolved within two days but reappeared on day 6. He was readmitted approximately 10 days after initial TMP-SMX treatment with fever, rash, and shortness of breath. On readmission, his physical exam was notable for fever of 39.4 degrees C and a pruritic full-body morbilliform erythematous rash including the palms and soles, and again sparing the mucus membranes. Results: Laboratory work-up showed eosinophilia (1200 per microliter; 13.9%) with an elevated C-reactive protein of 18.7. ESR, hepatitis panel, liver function tests, Lyme screen, rapid plasma reagin were normal. HIV was non-reactive. EBV testing was consistent with a past infection. ANA, dsDNA, and histone antibodies were normal. Chest X-ray revealed small bilateral pleural effusions. Urinalysis, respiratory viral panel, throat culture and blood cultures were negative. He was started on a very slow taper of prednisone for early-onset DRESS. On follow-up, rash had not recurred and eosinophilia had resolved. Conclusion: In patients with recurrent rash and multiple system involvement, DRESS should be considered. Early-onset DRESS should be in the differential in cases consistent with the syndrome but with presentation of symptoms earlier than expected, and after exclusion of other etiologies. Introduction: Erythema multiforme (EM) is an immune-mediated hypersensitivity reaction typically presenting with cutaneous and mucous membrane involvement. EM is known to be associated with exposure to drugs and viral pathogens. The most commonly associated pathogen is herpes simplex virus (HSV). However, there are reports of EM in patients diagnosed with Mycoplasma pneumonia. We describe a case of oral EM, a distinct but less well-recognized variant of EM, presenting as isolated oral mucositis in a patient with Mycoplasma pneumonia. Clinical Case: A 10 year old previously healthy male presented to his pediatrician with fever, cough, and lip swelling. Crackles were found on exam and he was prescribed azithromycin for treatment of atypical pneumonia. His facial swelling spread and he presented to the emergency department. He had buccal mucosal lesions with sloughing of oral mucous membranes without skin rash. There was concern for Stevens Johnson Syn-drome in response to azithromycin, so it was stopped. Symptoms progressed and he was seen in allergy/immunology clinic 1 day later where he was thought to have herpetic stomatitis and was started on valaciclovir. Oral swab for HSV-1/2 was sent. He was seen 2 days later when he had hives. He continued to have oral mucositis with improving facial swelling. His HSV-1/2 PCR were negative, so valaciclovir was stopped. Mycoplasma IgG and IgM were sent. After consultation with dermatology, his mucositis, which was characterized by extensive ulcerations covered with a pseudomembrane on the buccal mucosa, labial mucosa, tongue, and palate as well as with bloody crusted lip ulcerations, was determined to be diagnostic of oral EM. Positive Mycoplasma IgM and IgG titers confirmed the infection as the trigger for oral EM. He was treated with topical and systemic steroids with rapid improvement. Conclusion: Oral EM is a unique and much less described variant of EM. It is most often triggered by HSV infections, less commonly by drug reactions, and rarely by Mycoplasma infection. Unlike acute herpetic stomatitis, which involves keratinized mucosa and which can be confused with oral EM, our patient developed lesions only on nonkeratinized tissues. The triggers of oral EM and its characteristic presentation are important to recognize as repeated attacks often result in more severe forms of EM involving both the oral mucosa and skin. Background: Immediate hypersensitivity reactions (IHR) to low osmolar radiocontrast media (RCM) have an overall reported incidence of up to 3%. Pre-medication regimens reduce IHR rates in subsequent studies requiring RCM. However, breakthrough IHR (BIHR) may occur despite the use of premedication and low osmolar RCM. Consensus on successful pre-medication protocols for patients requiring RCM after BIHR is lacking. Case Report: A 50 year old male with a history of asthma presented for elective cardiac catheterization for unstable angina. He reported one previous RCM reaction with sneezing, hives, and ocular pruritis. Prior to catheterization, the patient received a modified Greenberger pre-medication protocol with oral prednisone 50mg (13, 7, and 1 hr pre-procedure), oral diphenhydramine 50mg and methylprednisolone 125mg 1 hour pre-procedure. Despite pre-medication, the patient developed generalized urticaria, periorbital angioedema, nasal congestion, and wheezing upon administration of a 3cc dose of low osmolar iohexol (640 mosm/kg). The procedure was immediately terminated and albuterol, diphenhydramine, and methylprednisolone were administered at that time and for two days post procedure. As the patient still required catheterization, a subsequent pre-medication protocol was implemented: prednisone 80mg daily, montelukast 10mg daily, cetirizine 10mg and famotidine 20mg twice daily for four days prior to the subsequent study. Additionally, he received methylprednisolone 125mg, diphenhydramine 75mg, and albuterol 1 hour pre-procedure. The patient underwent second cardiac catheterization one week later with successful placement of two drug-eluting stents using lower osmolar iodixanol (290 mosm/kg) with-out any adverse effects. Discussion: Breakthrough IHR to RCM are reported to occur at a rate of 0.11-18% and patients may experience symptoms comparable to their index reaction. Successful pre-medication regimens to prevent repeat BIHR in patients requiring utilization of RCM for subsequent imaging studies are not well documented. We describe a regimen which allowed our patient to receive critical coronary intervention. Further investigation into effective pre-medication in patients who successfully receive RCM may elucidate optimal regimens which may be most beneficial. S. Draikiwicz *1 , S. Arakali 2 , E. Capitle 3 , 1. Scotch Plains, NJ; 2. New Brunswick, NJ; 3. Newark, NJ. Adverse reactions have been reported in up to twenty percent of all patients receiving IVIG infusion.1 From October 1997 through July 2007, there were 10 reports of stroke, 6 reports of thrombosis, 4 reports of myocardial infarction, 2 reports of pulmonary embolus and 1 report of transient ischemic attack suspected of being associated with IVIG.2 While multiple known factors such as hypertension and diabetes contribute to the development of coronary artery disease and myocardial infarction (MI), it is also important to acknowledge the association of IVIG and post-therapy myocardial infarction. Our case is a 48 year-old man with a history of diet-controlled hypertension, steroid induced diabetes mellitus and polymyositis who developed a MI following IVIG. The patient was diagnosed with polymyositis in August 2013. His initial CPK was 10,000, elevated ANA titer at 1:640, and with a positive single-stranded DNA antibody. His polymyositis was refractory to multiple therapies including prednisone and mycophenolate mofitil. He was then started on IVIG therapy. He completed a three day course of IVIG from July 7 through July 10. On the morning of July 16th, he developed severe, constant, non-radiating chest pain. He presented to the ED more than 12 hours later and was diagnosed with a myocardial infarction. He had no history of cigarette smoking or prior cardiac history. His admission troponin T was elevated to 7.29 and he had a CPK of 2315. Cardiac catheterization revealed a 100% occlusion of the mid left circumflex artery for which he underwent angioplasty. We believe that IVIG caused a MI in this patient due to his lack of prior cardiac disease, relatively short term course of steroid induced diabetes mellitus (approximately 6-12 months), and a time course consistent with prior reported thromboembolic events caused by IVIG.3 This case is important because the application of IVIG continues to grow and providers need to be aware of the possible risks associated with this therapy. Introduction: Granulomatosis with Polyangiitis (GPA) is an antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAVs) with hallmark features of necrotizing granulomatous inflammation and pauci-immune vasculitis in small-and medium-size blood vessels. It has a predilection for the respiratory system and kidneys. GPA is extremely rare in children and adolescents especially of African American descent. Here we report a case of Granulomatosis with Polyangiitis in an African American teen. Case Report: 13 year old black boy born and lived in Grenada, in his usual state of health began to experience productive cough, blood tinged sputum, dyspnea, chest pain, malaise, and intermittent fevers. Chest x-ray (CXR) showed bilateral infiltrates, cystic changes and a 2.3 cm RUL cavitary lesion. Intradermal tuberculin test as well as sputum and gastric aspirate for acid fast bacilli (AFB) and HIV / HTV antibody were negative. Despite a 2 weeks course of antibiotics and 18 months empiric treatment for tuberculosis in Grenada his symptoms persisted. 17 months after initial symptoms he demonstrated microscopic hematuria along with rising serum creatinine. Five months later, in USA, his chest CT revealed a large thick walled cavity with an air fluid level in the RUL, tree and bud formation and a thin walled cavity in the left upper lobe. Laboratory investigation was significant for serum BUN / Creatinine of 65/4.1, positive ANA, and anti-Proteinase 3 .Renal biopsy revealed pauci-immune diffuse sclerosing and cresent glomerulonephritis with severe tubular atrophy and interstial fibrosis. He was diagnosed with GPA and started on high dose steroids and cyclophosphamide. Relapses were treated with rituximab. Currently he has hemodialysis dependent end stage renal disease, hypertension, dilatation of coronary artery, pericardial effusion required pericardiocentesis, concentric left ventricular hypertrophy, hypogammaglobinemia secondary to chemotherapy, on IVIG infusion, bilateral cataract secondary to chronic use of prednisone and posterior reversible encephalopathy syndrome. Conclusion: GPA is extremely rare in children and aldolescents especially of African descendant. Attributing symptoms of GPA to more common but less harmful disease processes, especially in areas of the world where it is less prevalent may occur. Earlier diagnosis and treatment is extremely important in preventing permanent renal damage. Introduction: Acquired angioedema with C1 inhibitor deficiency is a rare disease characterized by recurrent episodes of angioedema without urticaria or pruritis. Attacks may be triggered by trauma, psychological stress, or infection, but may also occur without an identifiable trigger. The etiology of ACID is frequently due an underlying malignancy (lymphatic or adenocarcinoma), a monoclonal gammunopathy of uncertain significance or an autoimmune condition. Case Presentation: A 70 year old female was referred for evaluation after a recent onset of recurrent episodes of angioedema without urticaria. Her initial episode developed in her lip approximately 24 hours after cataract surgery. A subsequent episode of angioedema occurred in her neck and jaw after a respiratory tract infection. Treatment with anti-histamines and corticosteroids did not improve symptoms. Blood work revealed a C4 <5 mg/dL (normal 10-40 mg/dL), C1-INH function=23% (normal >41%), C1-INH protein=6 mg/dL (normal 21-39 mg/dL) and a C1q < 3.6 mg/dL (normal 5.0-8.6 mg/dL) and a diagnosis of ACID was made. The patient was started on Aminocaproic acid 1gm TID to prevent attacks and Icatibant to treat acute attacks. To evaluate for a possible underlying malignancy, the patient had a CBC, lymphocyte profile, and a MRI of her abdomen, all of which were unremarkable except for a mildly elevated B-cell number at 482/cu mm (normal range 75 -375). Her ANA titer was 1:320 (normal 1:40). As part of the evaluation a SPEP and immunofixation was performed which revealed an IgG at 438 (normal 700-1600 mg/dL), and IgA 59 (normal 70-400 mg/dL), and normal IgM 81 (normal 40-230 mg/dL) with a polyclonal pattern. On review of symptoms, the patient reported 2 episodes of pneumonia, confirmed on CXR. Specific IgG to pneumococcal antigens was detectable for 7/14 serotypes, which decreased 5 months later (to 6/14 seropyes) after Pneumovax administration. Conclusion:To our knowledge, this is the first reported patient with ACID and a specific antibody deficiency Introduction: Severe combined immunodeficiency (SCID) syndromes are primary immunodeficiencies that result from the absence or diminished function of T cells and are classified by the presence or absence of B and NK cells. T-B-NK+ SCID occurs with autosomal recessive genetic defects involving V(D)J recombination or DNA repair mechanisms necessary to produce diverse T and B cell receptors and ultimately mature T and B cells. We present a case of T-B-NK+ SCID presenting with microcephaly, growth retardation, hypogammaglobulinemia, and recurrent infections. Case: An 18 month old female with a history of a multicystic, dysplastic right kidney, microcephaly, failure to thrive, and eczema presented with hypogammaglobulinemia identified while inpatient for a recurrent sinopulmonary infection and chronic diarrhea. History was significant for oral candidiasis at 8 months of age, sinusitis, recurrent acute otitis media, and diaper dermatitis since 16 months. Physical examination was notable for a small female with microcephaly, and xerotic, excoriated skin on the trunk and lower extremities, as well as inguinal erythroderma. Previous laboratory evaluation was significant for lymphopenia on CBC, undetectable IgG, IgA, and IgE, low IgM, and stool culture positive for yeast and rare Pseudomonas aeruginosa. Results: Repeat analysis confirmed hypogammaglobulinemia and lymphopenia. T, B, and NK cell quantification revealed low T and B cell counts with an increased number of activated, memory T cells and NK cell numbers that were low normal. T cell receptor analysis showed a polyclonal V beta repertoire for both CD4+ and CD8+ T cell subsets. The patient's lymphocytes did not proliferate in response to mitogens, confirming a diagnosis of T-B-NK+ SCID. Genetic testing revealed a previously reported H282R missense mutation and a novel L242IfsX5 frameshift mutation in the LIG4 gene producing a truncated DNA ligase IV protein. Conclusion: Hypomorphic mutations in DNA ligase IV are a rare cause of radiosensitive, SCID that can have a delayed clinical presentation from classic forms. Patients with LIG4 mutations typically have microcephaly and growth retardation, but can have a wide range in phenotype from mild immune deficiency to embryonic lethality depending on the residual function of the DNA ligase IV protein. Introduction: Mutations in glucose-6-phosphatase catalytic subunit 3 (G6PC3) are characterized by syndromic and non-syndromic severe congenital neutropenia. The syndromic form is associated with a wide spectrum of clinical findings, including thymic hypoplasia, primary pulmonary hypertension, cardiac, urogenital, and musculoskeletal abnormalities. Dursun Syndrome, a rare condition characterized by familial primary pulmonary hypertension, leukopenia, and atrial septal defect (ASD), is in the spectrum of G6PC3 deficiency. To date, over 60 patients with G6PC3 mutation have been reported. Here we present a patient with G6PC3 mutation associated with severe congenital neutropenia, lymphopenia, pulmonary hypertension, ASD and Poland syndrome. To our knowledge this is the first reported case of G6PC3 mutation and Poland syndrome. Case Report: A 3-year-old boy from non-consanguineous parents of Dominican descent with leukopenia and recurrent infections presented to our clinic at 8 months of age. He also has Poland syndrome (associated with absence of left pectoralis muscle, shortened left upper extremity, and syndactyly of the 3 rd and 4 th fingers of the left hand), a large secundum atrial septal defect, pulmonary hypertension, chronic lung disease, failure to thrive, aspiration, grade III vesicoureteral reflux, hydronephrosis and an inguinal hernia. He has had recurrent pneumonia as well as cellulitis and skin abscesses caused by S. aureus (MRSA), P. aeruginosa, E. coli, S. maltophilia, and E. cloacae. These illnesses have prompted many hospitalizations including ICU admissions for septic shock. Laboratory analysis is summarized in Table 1 . Genetic analysis revealed a homozygous G6PC3 mutation c.218+1G>A which was previously reported. He continues to have recurrent skin infections requiring hospitalizations despite filgastrim treatment. Discussion: Dursun Syndrome is a rare condition recently linked to G6PC3 deficiency associated with severe congenital neutropenia. Here we report a case of G6PC3 deficiency, Dursun syndrome, and Poland syndrome. Our patient's findings emphasize the wide clinical spectrum of G6PC3 deficiency. G. Imperato *1 , A. Khokhar 2 , S. LaBarba 1 , P. Ponda 1 , 1. Great Neck, NY; 2. Manhasset, NY. Introduction: Streptococcus pyogenes pharyngitis causes an associated exanthem in a minority of cases. Here we report a patient with an atypical prolonged and recrudescent exanthem secondary to Streptococcus pyogenes infection. Methods: A 51 year-old Hispanic female with no significant past medical history presented for evaluation of an erythematous and pruritic rash on her bilat-eral arms and legs. The rash first appeared approximately four weeks previously; six days after the rash appeared, the patient presented to a local emergency department for evaluation and was prescribed benadryl and instructed to be evaluated in an allergy clinic. Over the next three weeks, the rash gradually resolved with benadryl, and subsequently recurred four days prior to her initial clinic visit. The patient reported no history of food, environmental, or seasonal allergies. The patient works as a housecleaner, and reported a history of contact exposure to various household chemicals on regular a regular basis. Review of systems was positive for throat soreness which preceded the appearance of the rash. Physical examination revealed 1-2 cm erythematous blanching macules on the bilateral lower extremities with sparing of the soles, with scattered overlying excoriations. Erythematous papules were noted on the bilateral upper extremities, with a follicular eruption noted on the back. Areas of periungual desquamation (not present during the initial episode of rash) were noted on the hands bilaterally. Results: Laboratory studies included a comprehensive metabolic panel, as well as a complete blood count with differential, both of which were within normal limits. An anti-streptolysin O screen was elevated at 1220 IU/mL (ref 0-408 IU/mL). A throat culture grew Streptococcus pyogenes and mixed upper respiratory flora. The patient was treated with a five-day course of amoxicillin; her rash gradually improved but was still present ten weeks following its initial appearance. Conclusion: Streptococcal infection may cause prolonged and recrudescent exanthem in affected patients. While a range of diagnoses may be considered, Streptococcal pharyngitis should remain a leading suspicion for both adult and pediatric patients with atypical dermatologic findings when there is a clear clinical history of antecedent pharyngitis. S. LaBarba * , P. Ponda, Great Neck, NY. Rationale: Attention Deficit Hyperactivity Disorder (ADHD) is characterized by hyperactivity, impulsivity and/or inattention. ADHD affects 8-10% of all children, making it one of the most common childhood disorders. In the 2011 National Survey of Children's Health, the prevalence of ADHD in U.S children ages 4-17 years was 11%. This is a 42% increase from a prevalence of 7.8% in 2003. Up to 70% of children with this diagnosis have persistent symptoms into adulthood. Currently, ADHD is treated with 3 different types of medications: phenylphenidates, amphetamines and "other" class. These medications work to various degrees to inhibit norepinephrine and dopamine reuptake, thereby increasing the concentration of these neurotransmitters in the synaptic cleft. This in turn improves executive functioning, motivation, and reward perception. We describe three patients who had delayed reactions to different classes of ADHD medications. Methods: A 9 year old boy with ADHD was started on amphetamine and developed diffuse urticaria one week later. The rash resolved within two days of stopping the medicine. He then tried methylphenidate and lisdexamfetamine dimesylate respectively and developed urticaria one week after starting each medication. The rash resolved one day after stopping the medication on both occasions. Skin prick testing to all three drugs was negative. The patient was then placed on guanfacine and tolerated this drug without reaction. A 5 year old boy developed diffuse urticaria one week after starting guanfacine. This resolved two days after the medication was discontinued. A 34 year woman developed generalized pruritis five days after starting bupropion for ADHD. This resolved one day after stopping it. She then started lisdexamfetamine dimesylate and developed generalized erythema, pruritis, and sensation of throat closure one week later. These symptoms resolved after this medication was discontinued. Conclusions: Given the increasing frequency of ADHD in the U.S. population and the persistence into adulthood, it is important for clinicians to recognize ADHD medications as a possible source of delayed cutaneous reactions. Therefore skin testing may not be predictive in these cases. Both IgE mediated and non-IgE mediated reactions to these medications should be considered when the appropriate clinical history is elicited. Rationale:Eosinophilic cystitis (EC) is an inflammatory condition, often misrepresented as a bladder tumor due to its presenting symptoms of gross/microscopic hematuria, urinary frequency, dysuria and suprapubic abdominal pain. It is a rare condition seen in children and is thought to be caused by antigenic stimulation of IgE-mediated activation of eosinophils causing mast cell degranulation. This accounts for much of the inflammation and fibrosis to the bladder wall. We review our experience of treating patients with this condition in our allergy clinic. Methods:Two children with a history of acute onset of urinary frequency, suprapubic abdominal pain and gross hematuria were referred to allergy with the diagnosis of eosinophilic cystitis for the evaluation of potential allergens as a cause of eosinophilic cystitis. Results:Our patients include a 4 year old female and a 17 year old male who present with a history of hematuria referred for evaluation after routine urine and microscopy showed > 50 RBC's/high power field. Urine culture was negative. Both patients were diagnosed via cystourethroscopy with biopsy demonstrating a prominence of eosinophils with scattered neutrophils and lymphocytes. Allergic disease (asthma, allergic rhinitis, etc) was present in both patients. The 4 year old female had skin testing to common indoor and outdoor allergens which was negative. She was treated with antihistamines with resolution by 2 months follow-up. The 17 year old male did not have skin testing although immunocap testing showed egg IgE 38, milk IgE 52, peanut IgE 14, wheat IgE >100 and total IgE 1156. He was not started on any medications and had spontaneous resolution at 3 months follow-up. Conclusion: Eosinophilic cystitis is a rare inflammatory disorder of the urinary bladder. The exact etiology of eosinophilic esophagitis remains elusive though an allergic response has been proposed. Management comprises of removal of any suspected allergen, followed by antihistamine and corticosteroids. The course is usually benign and self-limited in children, although progression to fibrosis of the bladder with obstructive uropathy has been reported in some cases. Food Protein-Induced Enterocolitis (FPIES) is a non-IgE mediated food hypersensitivity that typically presents in infancy with vomiting and diarrhea. The diagnosis is made based on history and clinical presentation, and is confirmed by a supervised oral food challenge; there is no specific laboratory test to aid in diagnosis or in identifying the inciting food. Knowledge of potential trigger foods for FPIES thus is important in making the diagnosis, and subsequently counseling patients appropriately on food avoidance. In young infancy, the most common triggers are cow milk and soy formula. In older infants and children, FPIES may occur with ingestion of solid foods such as grains (most commonly rice and oat), meat and poultry, egg white, vegetables, fruit, legumes, and seafood. FPIES to cashew, however, has not been previously reported in the literature. In this report, we present the case of a 2.5 year-old boy who developed FPIES to cashew. His allergic history includes infantile FPIES to cow milk, which transitioned to development of IgE mediated allergy to cow milk at 9 months of age. After ingesting several cashews at 2.5 years of age, he had one episode of emesis. When skin testing with an allergist was negative for cashew, the mother was advised to reintroduce cashew. On re-introduction of cashew however, the patient had a reaction consistent with FPIES 2 hours afterwards: he developed repetitive emesis, lethargy, cyanosis, and diarrhea, notably without any cutaneous or respiratory symptoms. He was brought by ambulance to the emergency department for treatment, where he improved rapidly after administration of intravenous fluids. On repeat testing 3 months after this reaction, he was found to have small wheal in response to cashew on skin testing with diameter of induration 3 mm and diameter of erythema 0 mm. He also had mildly elevated specific IgE to cashew at 0.75 kIU/L. In conclusion, here we present the first reported case of atypical (older age of onset and association with detectable serum cashew-specific IgE) FPIES to cashew, adding to the list of potential allergic triggers for FPIES. Though rare, tree nuts such as cashew should be considered when identifying potential inciting food in children infants presenting with symptoms consistent with FPIES. Introduction: Dialyzer reactions refer to all of the abnormal sequelae resulting from the interaction between blood constituents and the hemodialysis (HD) membrane. There are two types of reactions: type A (0.004%) and type B (3-5%). In the past, these reactions were grouped under the term "first use syndrome" because they primarily occurred with new dialyzers. Here we present a case of hypersensitivity reaction during hemodialysis. Case: A 28 year old male was referred to the allergy clinic for diffuse urticarial rash during HD for the past 2 months. The pruritic rash starts within 2 hours of HD, on his face, chest and back sparing his lower extremities. The discomfort was such that he could not finish his 4 hours treatment. His Vitals remained stable and he denied any respiratory symptoms such as difficulty breathing, wheezes and cough. There was no angioedema, headache, blurry vision or GI complaints. He denied any history of medication, food, contact or environmental allergies. Past medical history: End-stage renal disease from IgA nephropathy, hypertension. Current medications: Procardia XL 30mg daily. Physical examination: He was alert, BP: 126/84 mm Hg, pulse 96/min, RR: 16/min. Cardiopulmonary and neurological examinations were normal. Skin revealed diffuse urticarial rash with blanching wheals on the face, trunk and upper extremities [Fig] . Methods: Laboratory data showed WBC: 5.2K/mm3 with 10% eosinophil. Serum tryptase level: 22.8mg/L (2hours after rash started); IgE-EIA: 44 U/ml, CH50: 57U/ml and C1Q binding: <1.2 Eq/ml. We suspected that the reaction was dialyzer related. We contacted the dialysis center and suggested that dialyzer be changed or reused. Prewashing the dialyzer with the patient's blood temporarily helped to alleviate his symptoms. We then prescribed Prednisone PO 40mg the night before, and 2 hours before dialysis. PO Benadryl 50mg just prior to dialysis. On follow up visit, the patient did very well with premedication, even when prednisone dose was tapered down to 10 mg. Conclusion: Common causes for dialysis resulted reactions include sensitivity to dialyzer. However, some patients continue to exhibit hypersensitivity reaction despite dialyzer changes. Premedication with steroid can successfully prevent the reactions. More studies are needed to better identify the cause of hemodialysis reactions in order to develop better management strategies to treat affected patients. Background: Angioedema causes self-limited, localized subcutaneous swelling especially likely to affect the periorbital and circumoral areas. Here we report a case of lip swelling in the context of angiotensin-converting-enzyme inhibitor (ACEi) use caused by extensive odontologic, maxillary and paraseptal abscesses. Case: A 44-year-old African American female smoker with a history of obstructive lung disease, atopy, hypertension on ACEi for 2 months, and diabetes mellitus type 2 was admitted to the hospital for her eighth pulmonary exacerbation in one year. She was treated with IV steroids and antibiotics, her second course of high dose IV steroids in six weeks. One week later, she reported sore throat and right ear pain, nasal discharge, pain in upper teeth, fevers, chills, neck tenderness and progressive swelling of nose, cheeks and upper lip. Allergy consult was made to evaluate patient's facial swelling. Results: Physical exam showed swelling of upper lip, nostrils and cheeks. Laboratory results significant for normal C1 esterase level but WBC elevated to 34,000. Sinus CT scan done 3 days prior to lip swelling showed a maxillary periapical abscess. Lip swelling and pain persisted for several days in spite of stopping ACEi and starting oral antibiotics. She then had spontaneous eruption of a large foul-smelling fluid collection from her nostrils. Subsequent maxillary-facial CT demonstrated cortical breakthrough of maxillary periapical abscess and interval development of septal abscess extending down into the philtrum with likely phlegmonous infection of the upper lip. She underwent I&D of septal abscess with aspiration of frank purulent material and placement of septal splints. She received more aggressive antibiotic treatment thereafter. Discussion: ACEi involvement is the culprit of the majority of drug-induced angioedema. However, angioedema that persists and worsens over time after ACEi discontinuation may point to other causes. In this case, a severe sinus infection with multiple abscesses led to inflammatory changes resulting in local facial swelling. Propensity toward development of this infection is likely multifactorial, including high dose steroids and poor oral hygiene, and protracted use of IV steroids likely made control of her sinus infection more difficult. R. Kreiner *1 , R. Rosenbaum 1 , A. Rubinstein 2 , 1. New York, NY; 2. Bronx, NY. Introduction: Anterior uveitis is the process of intraocular inflammation that results most commonly from a systemic immune-mediated disease. Methods: We evaluated a 9 year old previously healthy female who presented with anterior uveitis poorly responsive to conventional medical management and subsequently found to have an underlying familial immunodeficiency. Results: 9-yr-old female BZ was referred for immunologic evaluation after developing acute anterior uveitis presenting as red right eye, absent pupillary reaction to light and blurred vision. Initial diagnosis was made by ophthalmology on slit lamp examination. She was seen by multiple specialists including rheumatology for a suspicion of JIA as she briefly complained of joint pain. She also had a history of a mycoplasma pneumonia affecting several family members. BZ, however, had a persisting IgM response to Mycoplasma with a delayed class switch to IgG. At the onset of uveitis BZ was otherwise asymptomatic and afebrile. She was started on ophthalmic steroid drops for 6 weeks with resolution of symptoms and decreased signs of inflammation on slit lamp exam. Several weeks later she was found to have recurrent insidious inflammation. She was placed back on steroid ophthalmic drops with a marked improvement. Upon further analysis she was found to have low serum immunoglobulins. The family history revealed that her paternal grandfather and a paternal aunt suffer from Common Variable Immunodeficiency (CVID) who presented with recurrent infection and are being treated with periodic intravenous gammaglobulin (IVIG). BZ had no history of infections other than recurrent otitis in infancy. Conclusion: This is the first case report describing a patient with an underlying immune deficiency and family history of CVID. Her immune parameters do not completely fulfill the criteria for CVID. She presented with a persisting IgM to Mycoplasma infection with delayed class switch to IgG as well as an autoimmune uveitis. BZ responded well to topical and systemic steroids but the uveitis recurred promptly with a decrease of or discontinuation of steroids. She had a sustained response after 3 doses of IVIG at 1 gram/Kg and is now on prophylactic periodic treatment. Uveitis is a challenging disease as the differential is largely idiopathic. Early diagnosis is key as untreated uveitis can result in severely impaired vision. The IDEaL Patient Registry collects longitudinal information on subjects receiving immunoglobulin (Ig) replacement therapy from Coram CVS/specialty infusion services in an alternate care setting. Long-term outcomes and patient-reported quality-of-life assessments are not widely available for patients on Ig therapy for different disease types. In the IDEaL primary immune deficiency (PID) population, we examined baseline lab values for treated patients, and infection rates and quality-of-life assessments over time. Patients from our 140 investigators are eligible. IRB approval was obtained, as well as informed consent from all subjects. Information collected by Coram nurses and pharmacists was entered into the IDEaL database. Additionally, patients were asked to complete an SF-36 questionnaire and a Life Quality Index Questionnaire (LQIQ) every six months. Currently 310 subjects are enrolled in the Registry. The average age for diagnosis of adult patients was 59 years, and their average serum IgG level was 508 mg/dL. The average dose for subcutaneous Ig (SCIg) was 134 mg/kg/week; for intravenous Ig (IVIg), it was 465 mg/kg/month. In patients who switched from IVIg to SCIg, the average conversion ratio was 1:1.21. The average annual number of infections was three, with no significant difference between administration routes. Similar side-effect incidence rates, with differences in specific side effects depending on route, were noted. LQIQ results showed that most patients had a positive perception of their treatment, including how worthwhile and effective they felt it was, but were evenly split on how expensive they felt it was. These results from the IDEaL Registry suggest an overall profile of PID patients receiving Ig. Key findings include that adult patients were diagnosed later in life, SCIG dose was toward the lower end of the recommended dose range, and in patients switched from IVIg to SCIg, the dose conversion was 1:1.21. Patients appeared to have good infection control; however, with a limited dose spread, we could not demonstrate a strong dose response curve. Side effects were generally mild. Patients reported generally positive quality-of-life perceptions. Continued data collection will allow for further long-term data analysis on outcomes of patients on Ig treatment. B. Esquivel * , Mexico City, DF, Mexico. Aim: This study was aimed to determine the allelic frequencies of 22 related immune polymorphism (10488631, 1061170, 1265159, 12722489, 1799964, 1800629, 1800630, 2187668, 2476601, 3087243, 3135388, 3761847, 3890745, 4112788, 4140564, 6457617, 6822844, 6897932, 725613, 7574865, 800292, 9888739) in Mexican population and estimated the risk for immune diseases. These polymorphism have been commonly associated whit 9 immune diseases as; rheumatoid arthritis, macular degeneration, Type 1 diabetes, celiac disease, graves' disease, multiple sclerosis, lupus, osteoarthritis and psoriasis, and also are located in several of the most important immune genes as; HLA, STAT4, TNF, TNPO3, IL21, IL2RA, IL7R, MHC, C2, CFH among others. Method: Genotyping was carried out in 100 genomic DNA samples from dried blood spots supplied by the Mexican patients of Gene Test TQMedicine using custom made multiplex array in Sequenom Massarray System technology. Results: The frequency for all the polymorphism was calculated and previous risk alleles for autoimmune diseases determined in Mexican population. Our data shows similarities with those frequencies founded in human genome project and suggest an increase genetic probability for psoriasis and lupus in Mexican population. Patients with several risk alleles show an exacerbated immune response for several clinical conditions. For this patients that shown genetic risk for immune diseases, relevant clinical events were not observed since nutritional and clinical care were established to avoid the previous calculated genetic risk.. Conclusion: The Mexican population showed a slight frequency of immune associated diseases polymorphism, however those who have several risk alleles shown an exacerbated immune response. Follow up with immune modulators should be perform in this population to control this conditions. M. Stein 1 , R. Price 2 , A. Koterba *1 , W. Tuer 3 , 1. North Palm Beach, FL; 2. Palm Beach Gardens, FL; 3. West Palm Beach, FL. Reports from JS Rankin in the surgical literature suggest that cardiopulmonary bypass (CPB) may have immune depleting effects with some patients having low IgG levels. He reports that this in turn leads to pulmonary dysfunction which improves with intravenous immune globulin (IVIG). In our practice in the past 3 years, 3 patients with PID have undergone aortic valve replacements. One patient had significant post operative pulmonary problems. Case Reports: Case 1, 61 year old female with PID was receiving IVIG 25 gm every 5 weeks with trough IgG = 866 mg/dl on12/12/12. The next dose of IVIG was increased to 35 gm on 1/14/13. Aortic valve was replaced on 1/22/13, and she was on CPB for 87 mins. On 1/25/13 respiratory distress with a small infiltrate on chest X-ray leading to reintubation. On 1/29/13 IgG = 490 mg/dl and patient received IVIG 30 gm. There was subsequent rapid improvement over the next 48 hours. Two other females with PID had aortic valve replacement with no pulmonary or infectious complications. Case 2, 72 year old on 30 gm IVIG with trough IgG = 799 mg/dl and was on CPB for 85 mins. Case 3, 58 year old was on subcutaneous immune globulin 29.7 gm every 2 weeks. IgG trough was 1300mg/dl and she was on CPB for 64 mins. Conclusion: Reports from the surgical literature suggest that CPB may lead to a decrease in IgG. In one of three patients with therapeutic levels of IgG preoperatively, there was a significant drop in IgG after CPB and aortic valve replacement. Multiple factors could contribute to this drop including dilutional effects of intravenous fluids, active bacterial infection, and the use of CPB. The pulmonary infection and respiratory distress were accompanied by the decrease in IgG. A prospective study is needed in patients with and without PID, with measurement of IgG before surgery and 24,48 and 72 hours post CPB. At the present time it is important to consider whether there is a need for an extra loading dose of IVIG before surgery or whether careful monitoring of IgG levels after surgery will help assure that the PID patient will not have serious postoperative infections. Introduction: MWS is characterized by peculiar facial appearance, heart defects, Hirschsprung disease (HD), genitourinary anomalies and brain anomalies. It is caused by mutation of ZEB2 gene. Pons et al have described congenital hypo/asplenia in 4 patients with MWS and ZEB2 mutation, and suggest ZEB2 as a candidate gene for asplenia. Immune abnormalities have not been reported in MWS patients. Here we report two unrelated patients with MWS, ZEB2 mutation, congenital and/or functional asplenia and immune abnormalities. Methods: Chart and literature review Results: Patient 1 is a 6year-old boy with MWS, HD, heart defects, and congenital asplenia. He had no significant infection history. His immunological studies showed low IgG, IgA and IgM, but normal response to vaccination. T&B cell phenotyping showed normal percentages and numbers of mature B cells (CD19/CD20), but low percentages of CD19/27+ memory B cells. CD3 and CD8 T cell numbers were low but lymphoproliferative studies showed normal responses to mitogen and specific antigen. He lacked serologic evidence for past viral infection. In vitro cytotoxic-T-lymphocyte (CTL) function appeared normal with cellular ability to release interferon g in response to autologous EBV-transformed B cells (Elispot assay) and kill EBV-transformed B cells in cytotoxicity assay documented. In sum, no T cell defect was identified. Patient 2 is a 4-year-old girl with MWS, malrotation, hypogangolionosis, developmental delay, seizure disorder, and minimal splenic function from presumed splenic infarct. She had recurrent infections of URI, otitis, and sinusitis. Her immunological studies showed low IgG and IgA, and normal response to vaccination. T&B cell phenotyping showed normal percentages and numbers of T (CD3, CD4, CD8) and B (CD19, CD20, CD19/27+) cells including transitional and class-switched B cells. This child received empiric immunoglobulin supplementation and then antibiotic prophylaxis. Conclusion: Like the MWS patients reported by Pons et al, our 2 subjects also had congenital and/or functional asplenia associated with ZEB2 mutation. Both patients had evidence of immune discrepancies with low immunoglobulin levels. Physicians caring for patients with MWS should be aware of asplenia risk and possible immune abnormalities specifically B cell defects. Abdominal ultrasound and immune evaluation are recommended to reduce infectious morbidity. CVID is a collection of diverse and distinct diseases characterized by primary antibody deficiency (hypogammaglobulinemia). Clinical manifestations include autoimmunity, inflammatory disease, recurrent infections and increased malignancies, especially lymphoproliferative disorders. Inflammatory disease of the gastrointestinal tract is seen in about 50 % of patients and lymphocytic intraepithelial infiltration is common. Gastrointestinal infections, especially in patients with absent IgA are common and Giardia is a recognized culprit. We describe the case of a 21 year old male who was diagnosed with CVID at the age of 17 years after an episode of prolonged diarrhea and weight loss that required total parenteral nutrition. His quantitative Immunoglobulins were decreased (IgG 146 mg/dl (700-1600 mg/dL) IgA= <7 mg/dl (70-400 mg/dL) (IgM=<5 mg/dl (40-230 mg/dL)). He had protective antibody levels to measles, mumps, rubella and varicella but absent antibodies to tetanus vaccine. Replacement with intravenous immunoglobulins (IVIG) was started; because of low trough levels dose increases were necessary. A biopsy of his Duodenum showed complete absence of plasma cells, several non-necrotizing microgranulomata were found in gastric samples. Diphenoxylate and atropine was started for treatment of diarrhea. In the following the patient continued to have daily extensive diarrhea that prevented weight gain. Giardia was identified by stool culture. Despite prolonged treatment with various antibiotic regimens no eradication could be achieved. Treatment with Metronidazole, Tinidazole, Nitazoxanide, Paromomycin, Quinacrine alone and in different combinations was attempted for 2 years. Oral Budesonide and Loperamide were needed for control of his diarrhea on most days. Control of inflammation was also attempted with oral prednisone intermittently. Environmental precautions as avoidance of tap water were advised. A prolonged course of Quinacrine and Tinidazole cleared the infection and has kept him in remission for almost 11 months. Different components of CVID at interplay in the gastrointestinal tract required a delicate balance of controlling inflammation with corticosteroids and treatment of infection with antibiotics. Increased protein loss necessitated various dose adjustments of IVIG to allow adequate protection of reinfection. Chronic granulomatous disease (CGD) is an innate defect caused by mutations in any of the five subunits of the NADPH oxidase leading to impaired superoxide production and reduced intracellular killing of certain bacteria and fungi. Recurrent infections of the skin, lungs, lymph nodes, and liver are caused by a narrow spectrum of bacteria and fungi with particular susceptibility to catalase positive organisms. We report a case of Tsukamurella species pneumonia prompting diagnosis of p47 deficient CGD in a 17 year old male. A 17 year old Hispanic male developed cough, fever, and fatigue over one week. Cavitary necrotic lobar pneumonia of the right upper lobe, left lower lobe, and lingula was found on computed tomography. Sputum culture yielded Tsukamurella species and methicillin-sensitive Staphylococcus aureus. Prolonged treatment with meropenem and trimethoprim-sulfamethoxazole led to clinical improvement and resolution of consolidations. Past history included bacterial lymphadenitis at 18 months and 12 years, recurrent viral upper respiratory infections, and ulcerative colitis diagnosed at 17 years. In addition he had recurrent granulomatous skin disease with slow wound healing. Infection with Tsukamurella, an opportunistic organism, prompted immune evaluation. Dihydrorhodamine assay revealed abnormal oxidative burst pattern consistent with autosomal recessive CGD. Analysis of NCF1 showed a 2 base pair deletion (c.75_76delGT ) confirming the diagnosis of autosomal recessive CGD. Tsukamurella species are partially acid fast, aerobic, castalase positive, Gram-positive rods found in soil. Infections with Tsukamurella typically occur in immunocompromised hosts, particularly when neutropenia is present. Indwelling central venous catheter placement is also a risk factor. Cases of Tsukamurella have been reported in patients with severe combined immunodeficiency, leukemia, myelodysplasia, and in those receiving immunosuppressive chemotherapy. We report the first case of Tsukamurella infection in a patient with CGD. Introduction: Primary immunodeficiency diseases can have a variety of complications, mostly infections. We report a case with a renal complication. Case: A 26-y-o male presented with fever, pleuritic pain & productive cough for 1 d. He had adenotonsillectomy as a child and multiple pneumonias over the past 4 y. He appeared ill, in mild respiratory distress, T 1010F, RR 20/min, HR 118/min, BP 134-151/70-89 mmHg, Wt 97.5 kg, with dry mucous membranes, decreased breath sounds on the right lower lung field, and mild lower extremity edema. CXR showed RUL & RLL infiltrate with right-sided pleu-ral effusion. CBC: Hgb 10.1 g/dL, Hct 29.9%, Platelets 310 K/uL, WBC 20.12 K/uL, N 16.86 K/uL (84%), L 1.7 K/uL (8.4%), M 1.15 K/uL (5.7%), E 0.3 K/uL (1.6%). Serum protein was 4.5 g/dL, with albumin 1.1 g/dL. He was rehydrated with IVF & started on antibiotics (blood culture grew S. pneumoniae). His BUN 13 mg/dL & Cr 2.1 mg/dL increased to BUN 48 mg/dL and Cr 6.5 mg/dL by day 10. UA showed 500 mg/dL protein, Pr/Cr ratio 7.9 (nl <0.2). Renal biopsy demonstrated post-infectious glomerulonephritis (secondary to S. pneumoniae). Our evaluation showed IgG 102 mg/dL, IgM 31 mg/dL, IgA <8 mg/dL, IgE <0.3 IU/mL. He had nonprotective Ab titers to diphtheria (<0.1 IU/mL) & tetanus (<0.1IU/mL). Pneumococcal Abs were not done due to lab error. We administered IVIG 1g/kg. Upon obtaining his past medical records, we discovered that 3 y ago he had IgG 169 mg/dL, IgM 2 mg/dL, & IgA <5 mg/dL without intervention. At that time had normal BP and renal function. HIV test was negative. Flow cytometry was WNL. UPEP was consistent with non-selective proteinuria & SPEP was suggestive of acute inflammatory response. His stool α-1-ATP was negative. For glomerulonephritis, he was started on high dose steroid therapy with gradual reduction to 60 mg/day prednisone & anti-hypertensives. His findings were compatible with CVID complicated by nephrotic syndrome. IVIG continued (500 mg/kg) every 2-3 wk with peak 1280 mg/dL & troughs 370-662; and IgG half-life 6.3 d. His renal function fluctuated depending on his adherence to Lisinopril; with Cr 1.3 mg/dL & Pr/Cr ratio 2.3. He has remained infection-free with radiographic resolution of his pneumonia & pleural effusion. Conclusion: To our knowledge, this is the first reported case of CVID complicated with post-streptococcal glomerulonephritis possibly due to marked delay in IVIG therapy. A. Kleva * , A. Jongco, Great Neck, NY. Introduction: Sarcoidosis is a granulomatous disorder of unclear etiology that affects multiple systems. Monocytes, macrophages, and activated T cells increase production of inflammatory mediators in noncaseating granulomas. Peripheral lymphopenia correlates with disease severity. Methods: We report a relatively healthy 22-year old male with a history of psoriasis who presented with fever, cough, malaise after traveling to Cancun. He was diagnosed with bibasilar pneumonia and treated with levofloxacin. He was readmitted for fever, abdominal pain, diarrhea, hypoxia and neutropenia requiring filgrastim. Hospitalization was complicated by pneumothorax after bronchoscopy. Bronchoalveolar lavage showed Balantidium coli that was treated with doxycycline and metronidazole. After a few days, he was readmitted with fever, hypotension, hypoxia, diarrhea, and a new purpuric rash on the extremities. High dose steroids, imipenem, vancomycin and levofloxacin improved respiratory status and rash. He returned soon after discharge with pneumothorax needing a chest tube. As outpatient, prednisone was titrated to 40 mg daily without exacerbating rash or respiratory status. Bactrim prophylaxis was started. Results: At first readmission, T-cell lymphopenia was found (CD4+ = 83/uL, CD8+ = 35/uL). Bone marrow biopsy and extensive autoimmune and infectious workup were nondiagnostic. On readmissions, he had low IgG of 547 mg/dL. Open lung and skin biopsies showed a predominantly T-cell lymphohistiocytic infiltrate with CD4+>CD8+. Noncaseating granulomas were absent in both biopsies. As outpatient, lymphopenia persisted (CD4+ = 112/uL, CD8+ = 54/uL, CD19+ =6/uL, and CD 16+/56+ = 20/uL). Lymphocyte proliferation to PHA, IgG, IgA, IgM, CH50, and G6PD level were normal. He had protective titers to MMR, tetanus, diphtheria, pertussis and polio, but not to pneumococcus or varicella. A skin biopsy performed 6 months later showed noncaseating granulomas consistent with sarcoidosis. Lymphopenia persists (CD4+ = 82/uL, CD8+ = 32/uL and CD16+/56+ = 33/uL) while on weekly low-dose methotrexate, daily dapsone and prednisone 10 mg. Conclusion: Whether an underlying immune deficiency was present is unclear, but medication-induced immune suppression does not adequately explain his course. This case underscores the need for reevaluation of affected systems. Sarcoidosis should be considered in the differential diagnosis of lymphopenia. M.R. Shams * , L. Kobrynski, Atlanta, GA. Rationale: Whole exome sequencing (WES) is being used as a screening and diagnostic test for a number of rare diseases. The significance of mutations identified through WES requires interpretation by experts with knowledge of disease causing mutations. Methods: A 22 month old male presented to the Immunology clinic after WES revealed a mutation in GATA2. Genetic testing was performed because of a history of mild dysmorphic features, laryngomalacia, and obstructive sleep apnea. The only infectious history was recurrent acute otitis media requiring tympanostomy tubes and severe gingiva-stomatitis. Results: WES revealed a 3 distinct mutations; COMP R381H heterozygous mutation, TMCO C35Y heterozygous mutation and a de novo GATA2 R396W heterozygous mutation associated with known disease. GATA 2 defects are inherited in an autosomal dominant and cause increased susceptibility to severe viral infections, the development of mononuclear cytopenias, myelodysplastic syndrome/acute myeloid leukemia and lymphedema. GATA2 R396W has been previously reported in 2 patients, who developed symptoms in late childhood with infections with Epstein-Barr virus, herpes-simplex virus, human papilloma virus, mycobacterium avium intracellulare and the development of myelodysplastic syndrome. Our patient had a history of oral herpetic lesions due to herpes simplex but had no other significant viral infections. T, B, NK lymphocyte enumeration was normal. Neither parent had the R396W mutation. The other two identified mutations were carried by his father but are of unknown significance. Conclusions: WES is a useful testing modality to confirm suspected diagnoses. However, providers should use caution when interpreting genetic reports, especially if used as a screening modality, as many genetic variations are benign polymorphisms. It is critical to perform WES on both parents and, if available, affected family members to confirm the pathogenicity of a mutation. C.M. Duff *1 , M. Sher 2 , J. Leiding 2 , 1. Parrish, FL; 2. St. Petersburg, FL. Rationale: Immunoglobulin replacement therapy is prescribed for patients with immunodeficiency characterized by hypogammaglobulinemia and/or the inability to make antibodies to recall antigens. Administration can occur intravenously or subcutaneously. Currently there is a black box warning on all immunoglobulin replacement products indicating a possible increased risk of thrombogenic events. Data regarding the use of subcutaneous immunoglobulin (SCIG) therapy in patients with known thrombotic events is limited. Methods: A 36 year old Caucasian male with cystic fibrosis was diagnosed with selective antibody deficiency based on absent pneumococcal responses (0/14 protective serotypes) with intact tetanus and diphtheria responses and normal immunoglobulin profile (IgG 1020mg/dl, IgA, IgM). He is the survivor of a double lung transplant at 24 years and a single lung transplant at 33 years. A left leg deep vein thrombosis and sagittal vein thrombosis developed two years after second transplant. Causes of primary thrombophilia were excluded. In parallel recurrent bacterial pneumonias, primarily caused by S. pneumonia led to multiple hospitalizations. Based on poor antibody responses and clinical status, immunoglobulin replacement therapy was initiated. Results: To decrease the potential risk of a thrombotic event, 20% SCIG was administered more frequently at twice weekly. Concurrent treatment of thrombi continued with warfarin maintaining INR at a therapeutic level of 2-3 units. No bleeding or bruising occurred at infusion sites. A magnetic resonance angiogram performed 6 months after starting SCIG showed no increase in size or new thrombi. Protective pneumococcal titers were measured and were found to be protective to 10/14 serotypes and the patient has had only one hospitalization for bacterial pneumonia since starting SCIG. Conclusion: SCIG can be safely and effectively administered to patients with thrombi receiving anticoagulant therapy. Concurrent use of anticoagulant medications did not increase the occurrence of local site reactions with the use of 20% SCIG treatment in this patient. Introduction: CGD is a rare immunodeficiency of phagocytes characterized by increased susceptibility to bacterial and fungal pathogens. We describe the first report of two siblings with X-linked CGD due to the same mutation with significant variation in phenotype. Methods: A 6 week-old full term boy presented with fever, lymphadenopathy, and dyspnea. Imaging showed multifocal pneumonia. His hospital course was complicated by ARDS, septic shock, Candida lusitaniea fungemia, a cutaneous Candida and Rhizomucor abscess, multi-organ dysfunction, and HLH (Hemophagocytic Lymphohistoiocytosis). The "healthy" 18-month old brother of patient 1 was considered as a stem cell donor, but was found to have CGD caused by the same mutation. He was thriving with no major illnesses, but serological evaluation revealed systemic inflammation, normalizing on triple prophylaxis of Bactrim, Itraconazole and INFgamma. Results: In patient 1, Immunoglobulin B & T cells levels were normal, while NK cells were slightly low (92 c/mm 3 ). DHR showed absent neutrophil function: genetic testing revealed a hemizygous mutation in the CYBB gene of gp91 phox , consistent with X-linked CGD. After stabilizing medically, he underwent hematopoietic stem cell transplant (HSCT) from an unrelated donor complicated only by mild cutaneous GVHD. The second sibling had a negative DHR assay and the same mutation. ESR was >120, Plt 719, Hct 26.3, Alk Phos 310 and immunoglobulins were elevated. Screening CT showed 2-3 <1cm nonenhancing hepatic hypodensities, not amenable to biopsy. Lab abnormalities normalized within weeks of starting prophylactic therapy. At age 3, he remains free of recurrent bacterial and fungal infection. Discussion: CGD is caused by mutations in phagocyte NADPH oxidase, with considerable variability in disease severity. For many years, treatment consisted primarily of antimicrobial prophylaxis. Recently, HSCT has emerged as an attractive alternative, though there is debate about candidates for transplant. Generally, patients with very low super oxide production have worse long-term survival. Previous cases of siblings with AR-CGD have shown variable phenotype, presumably due to penetrance of the functional allele. Our cases are unique in that the patients have the same level of phagocyte function but very different phenotypes and highlight the potential difficulty in using level of super oxide production to assess patients for HSCT. H. Bhatti * , P. Poowuttikul, E. Secord, Detroit, MI. Background: Radiosensitive forms of SCID are due to defects in components of the NHEJ DNA repair mechanism during the process of V(D)J recombination. Mutations in several genes involved in the NHEJ mechanism result in a T-B-NK+ SCID. About 1/3 of patients with SCID have the T-B-NK+ phenotype, which is associated with a poorer prognosis after receiving hematopoietic cell transplantation in various studies. The components of DNA repair that are known to lead to radiosensitive SCID include Artemis, DNA Ligase IV, DNA-PKcs, Ku70, Ku80, XRCC4 and Cernunnos-XLF or NHEJ1. Patients with NHEJ1 deficient SCID typically have microcephaly with growth retardation; however there are a few cases who do not. Objective: We report the case of 21-month old female with a heterozygous NHEJ1 defect and T cell deficiency identified on newborn TREC screening. Case Report: A full term newborn was found to have very low TREC on newborn screening leading to suspected SCID versus a combined immunodeficiency. Exam of the baby revealed normal growth parameters and no dysmorphism. Subsequent testing revealed T and B cell lymphopenia (CD4 = 750/mm 3 , CD19 = 75/mm 3 ) with normal IgG and IgM with low IgA. Genetic testing revealed a heterozygous defect in the NHEJ1 gene. The lymphoblastoid cells revealed abnormal radiosensitivity of 15%. The T cell lymphopenia progressed until our last labs at 16 months. Testing for 22q11 deletion was normal. PHA was initially low but was adequate at 16 months and the child experienced no serious infection during that time. The family refused further care and the patient remains lost to follow up. Discussion: Deficiency of NHEJ1 is one of the variants of radiosensitive SCID. Five SCID patients reported to date have homozygous mutation of NHEJ1. Typically patients with this variant exhibit severe recurrent infections, microcephaly and growth retardation. Mild to severe T and B cell lymphopenia have been reported with progressive lymphopenia over time. We report a patient with heterozygous NHEJ1 deficiency in which the defect involves the dimerization domain, leading to a malformation of the functional enzyme similarly found in homozygous mutation. Our patient does not have microcephaly or growth retardation, but does show clear evidence of radiosensitivity and T cell lymphopenia, which we suspect will worsen with age. L. Buyantseva * , T. Craig, Hershey, PA. Background: Good syndrome is a rare cause of combined immune deficiency that occurs in association with thymoma. TACI and BAFF-R receptor mutations have been described in association with this syndrome. Signal transducer and activator of transcription 3 (STAT3) is a transcription factor essential for differentiation of the TH17 helper T cells. Mutation of STAT3 gene has been described in association with HyperIgM syndrome and a variety of autoimmune diseases. We present a case of STAT 3 mutation associated with Good syndrome. Case Presentation: A 73-year-old female with Common Variable Immunodeficiency (CVID) and thymoma removed 20 years ago. She presented with chronic watery diarrhea, 20 pound weight loss and frequent sinus infections. Her physical exam was remarkable for extensive onycomycosis. Immune workup showed lymphopenia, deficiency of B and T absolute cell counts (<1% and 58% respectively), normal NK cells count, and low IgA, IgG and IgM levels (<33 mg/dL, 6.7 mg/dL and 4.2 mg/dL respectively). Gastroenterology workup revealed celiac disease as a cause of her diarrhea. Gluten-free diet was initiated and her diarrhea resolved. Patient was started on Atovaquone for prophylaxis of opportunistic infections and IVIG replacement. Genetic analysis identified STAT 3 mutation. Conclusions: This is a first published report of a patient with Good syndrome associated with STAT 3 mutation. This case demostrates the variability of phenotypic responce to STAT 3 mutations. K. Achar *1 , D. Ferastraoaru 2 , D.L. Rosenstreich 2 , 1. New York, NY; 2. Bronx, NY. Introduction: Mannose binding lectin (MBL) recognizes carbohydrate patterns found on the surface of pathogenic microorganisms leading to activation of the lectin pathway of the complement system and contributing to ultimate pathogen elimination. MBL plays an important role in the first-line defense against Candida albicans and its deficiency is associated with herpes virus simplex (HSV) infection in mice. Method: Review of the literature and case report of a patient with MBL deficiency, esophageal candidiasis and recurrent oral herpes virus simplex infection. Results: 50 yo male with well controlled seasonal allergies was referred for evaluation of a possible immune deficiency after he was found to have one episode of esophageal candidiasis that resolved with oral fluconazole treatment. The patient did not have any other factors that could predispose to candidiasis, predisposing factors such as diabetes mellitus or treatment with oral or inhaled corticosteroids, and was HIV antibody negative. Further history revealed that he had been experiencing recurrent oral ulcers for the past 10 years the worst of which were over the lip, with more frequent and longer episodes for the past year. A repeat immunological evaluation revealed normal lymphocyte subsets, serum immunoglobulin levels, antibody responses (tetanus toxoid and pneumococcal titers), complement levels and a negative repeat HIV test. The serum MBL level on the first visit was low (12ng/ml ; normal: >100 ng/ml) and when repeated 3 months later was less than 0.5 ng/ml. The patient did not have any recurrence of esophageal candidiasis and he was prescribed acyclovir for recurrent oral HSV which reduced the frequency and severity of the oral lesions. Conclusion: This is the first documented case of associated esophageal candidiasis and recurrent oral HSV infection in a patient with MBL deficiency. The exact mechanism through which MBL deficiency predisposes to mucosal infections in patients with an otherwise normal immune system is not completely understood. We would like to highlight the importance of maintaining a high level of suspicion for MBL deficiency in these challenging cases. Making a correct diagnosis will help to prevent possible future complications by keeping these patients under closer observation. At the present there is no specific treatment available for MBL deficiency. L.I. Calderon * , A. Partida, B.E. Del Rio, O. Saucedo, C. Cicero, Mexico City, DF, Mexico. Introduction: Haemophagocytic lymphohistiocytosis is a life-threatening disease of immuneregulation. A large proportion of deaths occur early into treatment. We investigated association with early death for laboratory and clinical parameters. Methods: A total of 45 children from Federico Gómez Children's Hospital in Mexico city wich fulfill the diagnostic criteria and/or with familiar disease, receiving diferent treatments were included. We discribed demographic, clinical and laboratory data with frecuencies and proportions and also stadistics with central tendancy and dispersión tendancy. We made a risk analysis in two steps. The first was about an univariated análisis with proportional diferences in the survivor group and in non-survivers, using X 2 or exact Fisher test acording to the number of observations. From the variables that show statisticaly significant diferences, they were introduced in a model of multiple OR, in wich we also include time in months until clinical presentation, genus and etary group, getting a statisticaly significant model (LR X 2 (6g-l) = 13.32; p=0.038). Results: We observed a significant relation of risk for mortality for neutropenia (OR 8.96 IC95% 1.08 a 74.49, p = 0.042). The presence of clinical infection at the time of diagnosis show a relation with mortality without significant relevance (OR 7.17 IC95% 0.97 a 53.16; p= 0.054). The presence of hepatomegaly and absence of epistaxis are perfect predictors of surveillance. The surveillance curves point out the group between 1 and 4.9 years to be the more vulnerable with a higher mortality get in the first month after diagnosis of 50% and 70% thereafter. The group of less than 1 year show a mortality of 62%. The group of 5 to 10.9 years show a mortality rate of 66%. Finally the group of 11years and older, get a mortality rate of 55% at 3 months after diagnosis. Conclusión: In 45 patients with the diagnosis of haemophagocytic lymphohistiocytosis in the Hospital Infantil de México Federico Gomez, we found a mortality of 53%. The risk factor asociated to mortality was the presence of neutropenia. We also found a relationship with the presence of infection, but it wasn't statistically relevant. Moreover, in this study, we found posible protector factors related to mortality like the abscence of epistaxis and presence of hepatomegaly. Background: X-linked Agammaglobulinemia (XLA) is a primary immunodeficiency secondary to a mutation in the gene coding for the Bruton's tyrosine kinase (Btk) protein, which is essential for the differentiation of B cells. Immunological evaluation typically demonstrates a low/absent CD19+ B cells and agammaglobulinemia/severe hypogammaglobulinemia. The clinical presentation of XLA is usually between 6-18 months of age and is characterized by recurrent upper and lower respiratory tract infections gastrointestinal tract infections, conjunctivitis, skin infections/pyoderma, meningitis and sepsis. Ten to 20% of patients have neutropenia at the time of initial diagnosis. Case Description: An 18 month old male with a history of atopic dermatitis was admitted to hospital for a rash concerning for bullous impetigo. He had an absolute neutrophil count of 0/mm3 on admission which improved to 3500/mm3 after starting antibiotics for methicillin sensitive staphylococcal aureus (MSSA). Quantitative immunoglobulins were obtained revealing IgG<80 mg/dL (431-1109), IgA<6 mg/dL (20-100), and IgM<5 mg/dL . Vaccination titers to tetanus, diphtheria, haemophilus, and pneumococcus were absent. Flow cytometry on peripheral blood revealed no CD19+ B Cells and absence of BTK expression in monocytes. Conclusions: Although there are several proposed mechanisms for neutropenia in XLA, two non-mutually exclusive mechanisms have recently gained prominence. One proposed mechanism is that Btk is necessary for PMN function and development. In mouse models, neutrophils deficient in Btk have decreased expression of granular proteins (elastase, myeloperoxidase, gelatinase, and neutrophilic granular protein) and arrest of maturation at the myelocyte/promylocyte stage. Another proposed mechanism is that Btk negatively regulates cytokine and Toll Like Receptors (TLR) induced Reduced Oxygen Intermediates (ROI) production. In studies using human PMNs, a deficiency in Btk results in the overproduction of ROI during infection leading to increased neutrophil apoptosis compared to neutrophils from normals. However, neither of these mechanisms explains why neutropenia only occurs with infection prior to diagnosis and not subsequently following administration of replacement antibody. Regardless, XLA should be on the differential diagnosis of males between the ages of 6-18 months of age presenting with neutropenia. Hershey, PA; 3. Cincinnati, OH; 4. Budapest, Hungary; 5. Grenoble, France; 6. Milan, Italy; 7. Padova, Italy; 8. Tel Hashomer, Israel; 9. Cracow, Poland; 10. Jönköping, Sweden; 11. London, United Kingdom; 12. Barcelona, Spain; 13. Madrid, Spain; 14. Lisbon, Portugal; 15. Mainz, Germany; 16. Berlin, Germany; Germany; 18. Frankfurt, Germany; 19. Odense, Denmark. Introduction: Orally administered attenuated androgens (AAs) have been used since the 1970s for prophylactic treatment of hereditary angioedema (HAE) attacks, although they may be associated with dose-dependent undesirable side effects. Intravenous plasma derived C1-INH (C1-INH) has become available for prophylaxis in the last 5 years in some countries. However, long-term experience and safety data on this therapy is limited and its use varies by region and country. Our aim was to assess AA use across several international HAE centers and identify medical reasoning which may support switching patients from AAs to C1-INH prophylaxis. Methods: An 8-question survey exploring physician opinions on the use of prophylactic medications for HAE was sent to 17 HAE experts' in Europe (EU) and 3 in the United States (US). Experts reported on the percentage of patients receiving prophylaxis with AAs, side effects observed in patients receiving AAs and barriers preventing the switching of patients to alternative therapies. Results: The use of AAs varied among the HAE experts surveyed being most common in the EU compared to the US (Fig 1) , and was more common in men (27.6%) than women (13.4%). Other therapies used included C1-INH, antifibrinolytics and progestin (Fig 1) . Side effects reported in males receiving AAs included weight gain, lipid abnormal-ities and hypertension. Most reported side effects in females were menstrual irregularities, virilization and weight gain. A small number of patients discontinued AAs due to side effects (12%) while some patients (12%) switched to alternative treatments, e.g., C1-INH. Physicians reported that barriers preventing patients switching to prophylactic C1-INH included AA efficacy (n=10), ease of oral administration (n=10) and cost (n=7). Conclusions: The HAE expert survey demonstrates that AAs are widely used for prophylaxis in many countries. Experts noted that while AAs are effective they may be associated with side effects; however, at low doses (i.e., 50-100 mg/day) they may be well tolerated. In the absence of objective, comparative, randomized, controlled studies of the benefit/risks of new prophylactic therapies compared to AAs no consensus could be reached regarding switching patients from AAs to other prophylactic modalities. Introduction: Mutations of the GATA2 gene have recently been implicated as causes of MonoMAC syndrome, a primary immunodeficiency associated with monocytopenia and B and NK cell lymphopenia. The clinical ramifications are broad and include mycobacterial, viral and fungal infections, as well as pulmonary alveolar proteinosis and myelodysplasia. Long-term complications of the genetic defect include recurrent infections and likely leukemia. Case Presentation: A 33 year-old Hispanic woman presented to the emergency department with intermittent fever and a severe frontal headache of one week duration. Imaging revealed enhancing leptomeningeal nodules, and subsequent lumbar puncture identified cryptococcal meningitis. Her labs were notable for a white count of 6.3x10 3 and a monocyte count of zero. On further review, the patient's history was notable for interstitial fibrosis with pulmonary alveolar proteinosis, characterized by intermittent hemoptysis for the past 6 years. She also notes a varied infectious history, including Mycobacterium avium and Aspergillus fumigatus pneumonias as well as HPV infection with cervical intraepithelial neoplasia. A unifying diagnosis had not been offered in the past 6 years since she became symptomatic. There was no family history of immunodeficiency. Taking into consideration her disseminated fungal infection and monocytopenia, as well as the history of mycobacterial infection, PAP and HPV, GATA2 deficiency was suspected and genetic analysis revealed a frameshift mutation in the gene leading to MonoMAC syndrome. Conclusions: GATA2 deficiency is a relatively newly identified genetic mutation that can lead to a characteristic presentation of pathologic changes and infectious disease. Patients in whom this defect is suspected should undergo genetic sequencing to confirm the diagnosis. Ultimate treatment involves bone marrow transplant to prevent the likely long-term outcome of hematologic malignancy. North Palm Beach, FL; 3. Centennial, CO; 4. Atlanta, GA; 5. Galveston, TX; 6. Irvine, CA; 7. Vienna, Austria; 8. Westlake Village, CA. Introduction: IGHy allows for SC administration of IgG at a similar frequency (every 3-4 weeks) and bioavailability to that of intravenous (IV) IgG (IGIV). Compared with conventional SC-administered IgG (IGSC), IGHy requires fewer infusions per month (median 1.09 in the pivotal phase 3 study) using one or occasionally two sites. We report efficacy, safety and tolerability of IGHy in patients aged ≥16 years who were treated with IGHy for up to 3 years. Methods: Sixty-one patients aged ≥16 years received IGIV for 3 months followed by IGHy at 3-or 4-week intervals for approximately 18 months; 55 were treated for up to an additional 21 months at the same dose and interval. rHuPH20 was infused at 75U/g IgG followed by IgG at 108% of the IV dose. rHuPH20 was discontinued after up to 3 years of exposure; patients were followed for an additional 6-12 months. Assessments included rates of adverse events (AEs), serious AEs (SAEs), and infections; tolerability; and rHuPH20 antibody levels. These studies were approved by the appropriate ethics committees. Results: The maximum IGHy exposure for patients aged ≥16 years was 3 years (144 patient-years). Over the full study course, rates of temporallyassociated AEs/patient-year were 2.99 (local) and 2.78 (systemic; excluding infections). No SAEs were related to IGHy. The annual infection rate was 3.05 (95% CI: 2.63-3.52)/patient-year, including ramp-up. Of 2307 IGHy infusions administered (including ramp-up), 98% required no administration changes due to tolerability concerns or AEs. Thirteen patients ≥16 years developed nonneutralizing anti-rHuPH20 antibody titers ≥1:160 on 1 or more occasion with no associated AEs; titers declined despite continuing rHuPH20 in 12 of 13 patients. No patients developed neutralizing anti-rHuPH20 antibodies. Conclusion: In patients with PI who were treated with IGHy for up to 3 years, infection rates were low and AE rates were comparable to previously reported rates for patients treated with IGSC, but with infusion volumes and rates equivalent to that of IGIV. V. Cavero Chavez * , S.A. Schwartz, Buffalo, NY. Introduction: DiGeorge syndrome (DGS) is characterized by defective development of the pharyngeal pouch system. The triad is: cardiac anomalies, hypoplasic thymus and hypocalcemia. Patients are divided in two subtypes: complete DGS, with total absence of thymic tissue that is a type of SCID and fatal within the first year of life unless treated, and partial DGS which usually have not severe variable immunologic defects. The typical deletion associated is 22q11.2. Methods: We describe a 26 year old male patient with partial DGS and Tetralogy of Fallot surgically corrected, who presented with new recurrent sino-pulmonary infections for the past 8 months requiring multiple courses of antibiotics. He underwent immunological studies including: Lymphocyte subset counts, lymphocyte mitogens an antigen stimulation test, quantification of specific antibody titers for S. pneumoniae pre and post vaccination, immunoglobulin levels. Results: Immunological evaluation revealed Lymphopenia: Absolute lymphocyte count: 487/mcl(NR:1400-3300), CD3:409/mcl(NR: 1000-2200), CD4:143/mcl(NR:530-1300), CD8:231/mcl(NR: 330-920), CD19:25/mcl(NR:110-570), CD56:53/mcl(NR:70-480). Mitogen stimulation of mononuclear cells revealed a normal response to Pokeweed, decreased response to PHA and CON-A. Antigen studies included positive responses to Tetanus and Streptolysine O antigens, decreased responses to MMR and Candida. Serum immunoglobulins included low IgG (396 mg/dL), low IgA (59 mg/dL), normal IgM (55mg/dL). Serum antibody responses revealed baseline S. pneumoniae titers: 8/23 non-protective and post vaccination S. pneumoniae titers: 7/23 non-protective. Tetanus toxoid, varicella and Polio antibodies, were protective. Karyotype 45, XY, der (19) t(19;22) (p13.3, q11.2), -22, no previously described. Conclusions: Our 26 year old partial DGS patient with complex chromosome abnormality presented with late onset of humoral deficiency, lymphopenia and impaired T cell function. He required immunoglob-ulin replacement and prophylaxis for opportunistic infections. In this particular case genetic heterogeneity may play a significant role in time of presentation, manifestations and severity of impaired immunity. More understanding of this genetic variations will help to better know about prognosis of Partial DGS patients and continue close follow up even during adulthood. Introduction: IgG4-related disease is a systemic malady that has been reported to involve multiple organ systems, especially the pancreas with plasma cell infiltrates. It has also been found to affect skin, lacrimal glands, salivary glands, thyroid, pericardium, aorta, lungs, kidneys, pancreas, liver, retroperitoneum, breast, and prostate. Regardless of which organ is involved, tissue biopsies show great histopathological similarities such as diffuse lymphoplasmacytic infiltrates, abundant IgG4 positive plasma cells, and extensive fibrosis. Methods: Case files of patient was reviewed. Literature reviewed was perofmed using PubMed searches with the terms "IgG4" and "abdomen". Results: A 53 year old African American female presented to her primary care physician with chronic abdominal pain. She was referred for specialty care and had unremarkable esophagogastroduodenoscopy and colonoscopy. Subsequent CT scan revealed finding a 28x28x33mm soft tissue mass in the right lower abdomen/pelvis mesentery anteriorly abutting several loops of small bowel. Exploratory surgery was performed with abdominal wall mass and partial small bowel resection. The pathological report of the mass showed a storiform proliferation of dense fibrous tissue with marked infiltration by plasma cells. Immunohistochemistry for IgG4 showed up to 60 plasma cells staining per high power field. There was an associated phlebitis with infiltration by plasma cells. Blood IgG4 level was increased. These findings are consistent with IgG4-related disease. Conclusions: IgG4-related disease is a disease that can involve a wide range of organ systems. The pathological findings of IgG4-related disease have striking similarities. IgG4-related disease has broad clinical heterogeneity with multi-system involvement. Differentiation of IgG4 related disease from malignancy and other autoimmune disorder along with more clinical insight into the management of this disease are needed. More details of the radiologic manifestations of this disease aided by clinical and laboratory findings maybe essential for the accurate diagnosis of this disease and avoiding unnecessary invasive procedures. Introduction: Common Variable Immunodeficiency (CVID) is a heterogeneous group of disorders characterized by low levels of serum IgG, IgA and /or IgM and with loss of antibody production. The diagnosis is most commonly made between the ages of 20 and 40 years of age but children and older adults can also be diagnosed with this condition. There are many known clinical manifestations associated with CVID that include acute and chronic infections, inflammatory and autoimmune disease as well as an increased incidence of cancer and lymphoma. For patients with inflammatory and hematologic complications, there is a risk of developing lymphadenopathy, splenomegaly, autoimmune cytopenia, and/or granulomatous disease. Splenectomy has been used in patients with CVID often in the case of refractory autoimmune cytopenia or malignancy. Aside from a recent European report, there is limited information as the medical reasons and outcomes have not been well defined. Methods: A retrospective chart review on patients seen at an Immunology clinic based a tertiary academic center. The IRB approved chart review encompassed patients seen over the last 20 years and includes 43 patients with CVID and a history of a splenectomy. Results: The most common reasons for splenectomy in our patient cohort included hypersplenism (n=14), ITP (n=14), AIHA (n=8), presumed lymphoma (n=2), staging of Hodgkins disease (n=2) and lymphoma (n=1). These were not mutually exclusive reasons. Twenty-five patients had resolution of their symptoms at some point after splenectomy for either hypersplenism, AIHA or ITP. There were 13 significant complications after splenectomy in this cohort, which included pulmonary hypertension (n=4), hepatopulmonary syndrome (n=2), sepsis/infection (n=3) fistulae development (n=2), liver decompensation (n=2). Conclusion: There are significant risks associated with splenectomy in patients with CVID. Splenectomy has been used in patients with CVID and its role needs to be re-examined. M. Mortezavi * , E. Weis, R.J. Looney, Rochester, NY. Introduction: Non-infectious complications are now the major cause of morbidity and mortality in patients with Common Variable Immunodeficiency (CVID). We describe the case of a young woman with CVID who has both granulomatous-lymphocytic interstitial lung disease (GLILD) and unilateral optic neuritis. Case Presentation: Our patient is a white female who was diagnosed with CVID at age 16 but was not treated due to lack of insurance. At age 26, she presented with headaches, difficulty walking and profound hyopgammaglobulinemia (IgG 75, IgA < 5, IgM 32). She had multifocal brain lesions, which on biopsy were non-specific lymphoid tissue. Her symptoms and lesions resolved after treatment with maintenance IVIG and oral corticosteroids. At age 28, she developed dyspnea, a restrictive pattern on spirometry and ground glass opacity on CT thorax. She underwent an open lung biopsy, which showed GLILD. All her biopsies stained negative for viral and bacterial agents, and for IgG4 plasma cells. She was treated with four weekly rituximab infusions for GLILD. She improved symptomatically, but a CT two months later showed several new lung nodules. Soon after, she developed complete loss of vision in her left eye. Ophthalmologic examination showed a bulging optic disc and MRI of the orbit showed enhancing optic nerve consistent with optic neuritis. She improved after three doses of 1 gm IV methylprednisolone and mycophenolic acid, but relapsed shortly after. MRI of the brain and spine did not show any evidence of a demyelinating central nervous system disease. She was treated with pulse dose steroids and infliximab. She has regained her vision and continues to do well as her steroids are being tapered. Conclusion: There is only one previously reported case of optic neuritis in CVID. Patients with CVID can present with granulomatous lesions of the lung, skin and other organs. Optic neuritis is usually seen in demyelinating neurologic disease, such as multiple sclerosis (MS). Patients with MS-related optic neuritis usually show a quick and sustained response to pulse treatment with steroids. Given the quick relapse after stopping steroids and lack of evidence for demyelinating disease, we extrapolated that her optic neuritis may be due to a granulomatous process. We employed infliximab due to its success in treatment of neurosarcoidosis and had positive results. Neutrophil and monocyte function is crucial for normal wound healing. GM-CSF enhances function of both neutrophils and monocytes, and has been shown to improve wound healing in neutrophil dysfunction patients. Use of GM-CSF in wounds of normal patients has produced mixed results. We present case of successful treatment of refractory post-surgical wound with lymphopenia but without any known neutrophilic defects. 18 month old male with truncus arteriosus s/p failed surgical repair and subsequent heart transplant developed refractory non-healing post-operative wounds. He had history of recurrent fevers with persistent Coagulase-negative Staph Aureus bacteremia, but otherwise negative bacterial, fungal and viral cultures. He received multiple courses of empiric broad-spectrum antibiotics as well as antifungals and remained afebrile while on therapy, but was unable to stay afebrile off antimicrobials. Prior to transplant he presented with initial IgG 249, IgA 53 and IgM 23. He had hypoalbuminemia and high alpha-1-antitrypsin in stool(220 mg/dL) suggesting protein-losing enteropathy. He also had severe lymphopenia(200 cells/ml) with CD4 46 cells/ml, CD8 8 cells/ml, CD 19 25 cells/ml and NK 99 cells/ml. He received IVIG weekly to keep IgG>1000. PMN oxidative index was 60 ruling out Chronic Granulomatous Disease. Whole Exome Sequencing found compound heterozygous c.422C>G and c.6064T>C variants of uncertain significance in PRKDC gene. Mutations of this gene have been implicated in forms of autosomal recessive SCID due to defects in DNA repair. No mutations were found in any genes associated with neutrophil defects. GM-CSF aqueous solution was applied to 2 refractory surgical wounds 3 times daily over 2 weeks. Significant improvement in color, size and degree of healing was observed by multiple physicians and nurses. GM-CSF leads to enhancement of granulation tissue development in wound healing, and has been shown to be beneficial for wounds in neutrophil dysfunction patients. Very little data exists in its efficacy in patients with immunodeficiencies other than primary neutrophil defects. Given that T-cells are a significant source of GM-CSF, and the observed improvement in wound healing seen in this patient, use of GM-CSF ANNALS OF ALLERGY, ASTHMA & IMMUNOLOGY may be considered in refractory wounds in patients with severe primary or acquired lymphopenia. G. Drannik 1 , F. Gaisenyuk 1 , V. Driyanska 1 , N. Stepanova 1 , L.M. DuBuske *2 , 1. Kiev, Ukraine; 2. Gardner, MA. Introduction: Cytokines are key factors in pathological processes including urinary tract diseases. Urinary tract mucosa contains various protective effector molecules including antimicrobial peptides and inhibitory proteinases such as secretory leukocyte protease inhibitor (SLPI) produced by mucosa cells, macrophages and neutrophils which assists in defense against elastase secreted by activated neutrophils, and has antibacterial and anti-inflammatory properties. Levels of cytokines and SLPI in plasma and urine of the patients with chronic infections of urinary system (USCI) including cystitis and pyelonephritis may be critical in disease pathogenisis. Methods: Levels of interleukin (IL)-1, -4, -6, -10, TNF-, TGF-1 in peripheral blood of 12 patients with chronic cystitis ( 1 st group) and 25 patients with chronic pyelonephritis (2 nd group) were assessed by ELISA methods using STAT FAX-303 PLUS ("Immunotech", "Diaclon", France; "DRG", Germany and "Hycult biotechnology" for Human SLPI (Netherlands). Results: Patients in both groups showed high levels of cytokines compared with normal persons (p<0.05). Serum TNF-levels were greater in Group 1 99.6±6.5 versus Group 2 43.5±2.0 pg/ml (p<0,001) but the urine levels in Group 1 exceeded the levels in Group 2 by 3-fold (p<0.001); IL-1, IL-4, IL-6, IL-10, IL-17, IFN-γ, TGF-, though exceeding normal levels, did not differ between Groups 1 and 2. The SLPI level in urine from Group 1 was greater (4171±225) than in Group 2 (1857±203 pg/ml; <0.05). SLPI levels in serum of Group 1 (2052+378) and Group 2 (2240+178) were greater than normal but did not differ( 2240±178 versus 2052±316 pg/ml; P>0.05). Conclusion: TNF-, that was greater in cystitis than in pyelonephritis, The elevated SLPI-levels were seen in urine with levels >3200 pg/ml typical for the presence of cystitis. Cytokines and SLPI have an important role in immunity of the urinary bladder mucosa and antibacterial immunity in USCI. Background: Lipopolysaccharide (LPS) or endotoxin of Gram-negative enterobacteria may help to maintain autoimmune inflammatory responses in SLE. One indicator of the strength of immunity is antiendotoxin CD14 expression. Methods: 48 SLE patients were studied. Levels of potential of specific receptors to endotoxin (CD14) on granulocytes in peripheral blood were determined by laser double staining flow cytometry using monoclonal antibodies to anti-CD14-REIO-Test® (CD14). Levels of general Ig, IgM and IgG antibodies were assessed by ELISA. Circulating immune complexes (CIC) were determined spectrophotometrically by precipitation with polyethyleneglycol (PEG). Results: The levels of CD14 receptors in SLE patients on granulocytes was 18.57 ± 0.502 fluorescence units (UF). The level of total IgA was 2.041 ± 0.059 g/l, IgM was 1.927 ± 0.087 g/l and IgG was 11.34 ± 0.171 g/l. CIC level was 19.288 ± 1.546 conventional units (CU). In studying the relations of these parameters an inverse relationship between CD14 receptor level and the level of total IgA was found with R= -0.404 (Pearson correlation) at a significance level of p = 0.004. Dependence was described by the formula: IgA = -1.734 * CD14+3.258. There was also a direct significant correlation of receptors CD14 and CIC (R = +0.371; p = 0,009) expressed by the formula: CIC = 41.47 * CD14 -9.81. Significant relationships between CD14 and IgM and IgG antibodies were detected. Conclusions: An association of cellular anti-endotoxin immunity as represented by the CD14 receptor with humoral immunity (IgA, CIC) was seen in patients with SLE. This support s arole for LPS of Gram-negative intestinal flora in modulating the immune status of SLE patients. Background: Spermatozoa and leukocytes in semen may produce reactive oxygen species (ROS) which may have and importance in chronic abacterial prostatitis. Methods: Reactive Oxygen species can be assessed by using nitroblue tetrazolium (NBT). Levels of ROS can be measured by chemiluminescence. The NBT test was performed according to the method adapted to ejaculate. In the light microscope with an increase in x 100 counted stained percentage of neutrophils and spermatozoa in the ejaculate. To avoid additional procedures, determination of ROS in cells was performed using native ejaculate without using a density gradient. Cells that had large and small dark blue deposits occupying more than 50% of the sperm cytoplasm were called positive. Results: Based on leukocyte concentrations in semen,patients samples were classified into 2 separate groups: leukocytic -sperm group (leukocytic count > 1 x 10 6 peroxidase-positive leukocytes[PPL]/mL semen; n = 20) and non-leukocytic sperm group(leukocytic count 1 x 10 6 PPL/mL; n = 63). In Group 1 the NBTtest showed that the overwhelming number of patients (75%) had 60% of sperm producing reactive oxygen species (ROS), more than in healthy men with sperm ejaculate normally characterized by low ROS generation; percentage of NBT positive sperm is 15.5 ± 4.2%). Antioxidant properties of ejaculate based upon NBT test in 75% of patients was decreased by 90%. Group 2 had ROS production by sperm by NBT test in 75% of patients with 56% of sperm producing ROS. Antioxidant properties of the ejaculate by NBT test in 75 patients was decreased by 70%. Conclusions: The NBT test can be used to assess the contribution of seminal leukocytes and defective spermatozoa towards ROS generation in semen. In infertile patients with a chronic abacterial prostatitis production of ROS by seminal spermatozoa is increased and does not depend on the quantity of leukocytes in the ejaculate. Background: Obstruction of the upper urinary tracts results in the re-modulation of the tubulointerstitial tissue that is a precondition for nephrosclerosis. TGF-β1 influences each stage of development of tubulointerstitial and glomerular lesions. Levels of TGF-β1 in the urine of children with obstructive and refluxing megaureter (MU) may be critical in disease development. Methods: The TGF-β1 level in urine was assessed in 54 children (1 -9 years old) with obstructive (23) and refluxing (31) MU; with or without pyelonephritis (PN). The control group consisted of 10 healthy children. The TGF-β1 concentrations were assessed by the immuno-enzymatic method with "DRG" (USA) test system. Results: TGF-β1 concentration in urine taken from the urinary bladder of children with obstructive and refluxing MU statistically exceeded the values in the control group being 15.3 ± 4.35 pg/ml (P<0.001). The distribution of values in controls was 0 to 5.4 pg/ml, while in children with MU it was 8.8 -50,9 pg/ml. There was an increase in MU when compared with the control of TGF-β1 excretion in the patients with obstructive (15.7 ± 2.1 pg/ml) and refluxing MU (16.9 ± 3.8 pg/ml) (P<0.001); no difference was noted between the groups and was similar to the TGF-β1 excretion in urine of groups of MU patients complicated by pyelonephritis (16.6 ± 1.6 pg/ml) (P<0.05 versus controls). Conclusion: Increase in the TGF-β1 level above normal at the first examination, or continuous increase during examination every 3 to 4 months in comparison with the values of the first examination, are considered evidence of aggravation of the functional state of the kidney due to the active fibrotic processed in patients with megaureter. TGF-β1 plays a role in the pathogenesis of renal disease in these patients. ful treatment options. We present a 9 month old patient with recurrent lung abscesses who was successfully treated with an interleukin-1 antagonist. The case illustrates the importance of early diagnosis and treatment of autoinflammatory disorders. Case Report: A female patient presented in the newborn period with recurrent sterile skin abscesses that resolved after antifungal and antibiotic therapy. She was then well until 9 months of age, when she developed recurrent sterile lung abscesses which were unresponsive to trials of antibacterial and antifungal treatment, but which resolved completely with Anakinra and Canakinumab therapy. Results: Laboratory testing revealed negative cultures with persistent leukocytosis, thrombocytosis, and elevated acute phase reactants. Lung biopsy was sterile and without granulomas, and lung parenchyma showed reactive type 2 pneumocyte hyperplasia, chronic interstitial inflammation, and detached acute inflammatory debris. The drained abscess showed evidence of acute inflammation and debris with 90% neutrophils, 12% macrophages, 4% eosinophils and 4% small lymphocytes. Skin biopsy showed neutrophilic folliculitis with organizing dermal abscess. Conclusions: The spectrum of clinical presentations of autoinflammatory disorders is growing. Low threshold for early treatment initiation with IL-1 antagonists is necessary in the setting of persistently elevated inflammatory markers and sterile neutrophilic biopsy without evidence of infectious etiology. B. Patel * , A.S. Nickels, G.W. Volcheck, A.Y. Joshi, Rochester, MN. Background: Diagnosis of CVID presents a diagnostic challenge given variable interpretation of the diagnostic criteria. Clinical findings are not included as part of the diagnostic criteria, however they can be helpful in further supporting or refuting the diagnosis. CVID is associated with many other diseases including granulomatous disease, though the mechanism of association is unclear. Case Presentation: 47 year old female presented with a progressive rash for 2.5 years, lymphopenia and hypogammaglobulinemia, though she denied an infectious history. Rash consisted of well circumscribed plaques with lichenification and mild scaling on the face and trunk. Biopsy of her rash demonstrated eosinophilic spongiosis and noncaseating dermal granulomatous inflammation with eosinophils. Staining for an infectious etiology was negative. Exam demonstrated axillary lymphadenopathy and follow up PET/CT demonstrated mild uptake in areas of thickened skin overlying the right breast and left axilla and multiple prominent right axillary lymph nodes. An excisional right axillary lymph node biopsy demonstrated non-necrotizing granulomatous lymphadenitis. Bone marrow biopsy and peripheral flow for lymphoma/leukemia were negative. An angiotensin-converting enzyme (ACE) level was elevated at 68. Blood work demonstrated a low IgG of 587 (reference range 767-1590mg/dl) and a low IgA of 50 (reference range of 61-356mg/dl) but normal IgM and IgE levels. She had a mildly reduced response to the pneumococcal vaccination as defined by the 2012 AAAAI working group statement, but she had protective titers to diphtheria and tetanus vaccination. A lymphocyte flow report demonstrated a decrease in the class switched memory B cells (CD27+M-D-). Conclusion: This case describes granulomatous disease as the primary presentation of possible CVID. This patient meets the least stringent diagnostic criteria for CVID, but interestingly does not have an infectious history. However, recurrent infections can develop over time in patients who meet criteria for CVID and would not be uncommon in patients who initially present with granulomatous or autoimmune disease. Therefore, this patient should be closely monitored for infection and consideration of treatment with IVIG. S.E. Henrickson *1 , S. Jyonouchi 2 , 1. Haddonfield, NJ; 2. Philadelphia, PA. Introduction: Two toddlers presented with skin abscesses, recurrent acute otitis media (AOM) and neutropenia with both weakly positive anti-neutrophil antibodies and ELANE mutations consistent with severe congenital neutropenia (SCN). Here we consider when it may be necessary to consider genetic testing even after anti-neutrophil antibodies are positive. Clinical Cases: Patient 1: 17-month-old female presented with neutropenia, delayed umbilical cord separation, recurrent AOM, oral ulcers and progressive, refractory skin abscesses. She was referred to Infectious Disease where an assessment yielded appropriate humoral immune responses, normal B and T cell flow cytometry and CBC with ANC 126. With persistent skin abscesses, she was referred to Dermatology and then Immunology. A repeat CBC with ANC of 0 led to urgent referral to hematology and BM biopsy with evidence of blockade in granulocyte development. She was had weakly positive anti-neutrophil antibodies and an ELANE mutation consistent with severe congenital neutropenia (SCN). Patient was started on G-CSF and required high dose G-CSF (15mcg/kg/day), with a full sibling match identified for BMT. Patient 2: 8-month-old male presented with a history of chronic AOM, perirectal abscess complicated by fistula formation and pneumonia requiring prolonged IV antibiotic therapy. Based on recurrent infections, he was referred to Immunology and labs showed persistent neutropenia, with referral to Hematology and a bone marrow biopsy with decreased granulocyte maturation, consistent with severe congenital neutropenia. He was found to have weakly positive anti-neutrophil antibodies and an ELANE mutation consistent with severe congenital neutropenia (SCN). He was started on G-CSF, requiring high doses (10mcg/kg/day), with only modest increases in his neutrophil count. He received an identically matched sibling bone marrow transplant. Conclusion: We have not identified any prior case reports of anti-neutrophil antibody positivity in patients who have SCN. Patients with autoimmune neutropenia do not generally have difficulty with recurrent infections. Therefore, In neutropenic patients with multiple, severe, recurrent infections at an early age and weakly positive anti-neutrophil antibodies, SCN should be fully evaluated. Introduction: Idiopathic CD4 Lymphopenia (ICL) is a disorder defined by persistently low CD4 T-lymphocyte count (CD4 < 300/mm 3 ) or CD4 count less than 20% of total lymphocytes on more than one occasion with no evidence of human immunodeficiency virus (HIV-1/2) or human T-cell lymphotropic 1/2 (HTLV-1/2) infection and no other secondary causes of immunosuppression. Other features include high serum IL-7 levels, but poor signaling through IL-7. ICL patients can present with autoimmune disease, malignancies or opportunistic infections. The most common infections include Cryptococcus, nontuberculosis mycobacterial infection and human papilloma virus (HPV). Very low CD4 counts (< 150/mm 3 ), low CD8 counts (< 180/mm 3 ; normal 212 -1007/mm 3 ) and low natural killer (NK) cell counts (< 100/mm 3 ; normal 97 -421/mm 3 ) are all associated with increased morbidity. Treatment options considered were immunomodulators such as interleukin-2 (IL-2), interleukin-7 (IL-7) and interferon-gamma. We describe a case of cutaneous HPV infection in ICL treated with interferon-gamma. Methods: Case Report Results: A 34 year-old female with known ICL presented to Immunology clinic with digital warts and severe plantar warts on her left sole which were scraped monthly. Her previous treatment with IL-7 was ineffective. Warts were unresponsive to Imiquimod and Urea 40% gel. Laboratory results were significant for a very low CD4 count at 18/mm 3 , low NK cells 86/mm 3 , low CD8 count 69/mm 3 . Mitogen and antigen-induced lymphocyte proliferation profile was decreased to candida, tetanus toxoid, tuberculin purified protein derivative (PPD), phytohemagglutinin (PHA), pokeweed mitogen (PWM) and concanavalin A (Con A). Serum immunoglobulins and mannose-binding lecithin (MBL) levels were within normal range. We started treatment with Interferon-gamma-1b 100mcg three times a week for one month. Our patient's digital warts resolved and the plantar warts significantly decreased after one month of treatment with Interferon-gamma-1b. Conclusion: ICL is a rare immunodeficiency that can present with HPV infection. Severe cases of cutaneous HPV infection with very depressed T-lymphocyte counts may be unresponsive to topical or destructive therapy. However, treatment with immunomodulators such as Inteferon-gamma-1b may be promising. Rationale: The prevalence of lymphopenia, neutropenia and thrombocytopenia in children without HIV infection, malignancy, or aplastic anemia is rare. Frequent infections are the primary clinical manifestation. Methods: An 8 year old (yo) African American female with recurrent infections since 1 yo was monitored with serial CBC's, immunoglobulins, lymphocyte subsets, specific antibody titers, and bone marrow biopsies. Results: From 1 yo, this patient had recurrent otitis media, pneumonia, and skin abscesses. She had diffuse verrucae plana for the last 2 years. Serial CBCs showed intermittent neutropenia (ANC ranging 570-6248 cells/uL), marked lymphopenia (ALC 272-943 cells/uL), and thrombocytopenia (platelet counts 54,000-120,000 cells/uL). Lymphocyte subsets (CD3, CD4, CD8, CD19, and CD3-CD16+CD56+) were persistently very low, as well as low CD27+ B cells (memory B cells). HIV PCR, HIV-1/2 antibody, and Parvovirus antibody were negative. Neutrophil associated antibody was negative. Specific antibody titers to Streptococcus pneumoniae and Haemophilus influenzae b normalized following booster vaccines, with fairly good amnestic titers. Quantitative immunoglobulins were normal, until recent onset of low IgM levels. Lymphocyte response to mitogens and specific antigens were normal. Flow cytometry for paroxysmal nocturnal hemoglobinuria was negative. 1,2:3,4-diepoxybutane (DEB) clastogen assay for Fanconi anemia was negative. Chromosomal study showed 46, XX, with no clonal abnormality. Fluorescent in situ hybridization (FISH) for Myelodysplasia panel and DiGeorge (22q11 and 10p13p14) were negative. Bone marrow at 1 yo showed normocellular trilineage marrow with erythroid and megakaryocytic hyperplasia, and at 5 yo showed hypocellular marrow (60-80% cellularity) with trilineage maturation and mild 1+ fibrosis. Megakaryocytes were normal in number and morphology. Chromosomal microarray analysis showed duplication at 10q26.3, confirmed by FISH and a deletion at 11p11.12. She began daily trimethoprim/sulfamethoxazole prophylaxis 3 years ago, with very few bacterial infections since then. Conclusions: Patients with lymphopenia and frequent infections may benefit from antibiotic prophylaxis. Monitoring with appropriate lab tests can guide the treatment plan. Given this patient's onset of verrucae plana, additional evaluation for WHIM syndrome may be warranted. V. Nayima * , P. Atkinson, Birmingham, AL. Rationale: Nuclear factor-κB essential modulator (NEMO) deficiency is a combined immunodeficiency due to hypomorphic mutations in the X-linked IKBKG gene. Symptoms include antibody deficiency, susceptibility to nontuberculous mycobacteria, and ectodermal dysplasia. Female carriers of severe loss of function mutations in IKBKG have a condition called incontinentia pigmenti. Case description: A Caucasian male infant (36.5wga) presented to clinic at one week after circumcision with MRSA infection of the penis. Five weeks later he again presented for evaluation with thrush, severe eczema, bloody stools, and a submandibular mass that grew MSSA. At 12 weeks of age he was admitted for fever, diarrhea, and leukocytosis (WBC 40K) and was found to have significant eosinophilia ( 24%) and severe panhypoimmunoglobulinemia. He was treated with empiric antibiotics and IVIG. At 14 weeks the patient was admitted for weight loss and leukocytosis (WBC 45K) and was found to have a right inguinal abscess that grew MSSA and multiple bowel flora. He was readmitted at 5 months for a planned bone marrow transplant after positive genetic tests. He continued to have poor weight gain and experienced multiple infections during the pre-transplant interval with MSSA, MRSA, coagulase negative staphylococci. Results: Complement tests, CD11/CD18 protein expression, mitogen stimulation, neutrophil respiratory burst, FOXP3 protein expression, NK cell function, T and B cells and subsets including Th17 cells by flow, and HHV 6/7/8 PCR testing were all normal/negative. CD45 RO (naïve) CD4+ T cells were decreased by flow. During his evaluation it was discovered that his mother had an extensive patchy hyperpigmented birthmark that was consistent with incontinentia pigmenti. Gene sequencing revealed a hemizygous IKBKG gene (H413D) variant which has not been published as mutation or benign polymorphism, not identified in 1200 individuals with European/African American ancestry, and predicted to be pathogenic. Conclusion: This early and severe presentation of NEMO/IKBKG deficiency suggests that this diagnosis should be considered in very young infants with severe infections and persistent leukocytosis even in the face of largely normal immunologic testing. K. Cook * , Orange, CA. Introduction: Spice allergy is relatively rare accounting for approximately 2% of all observed food allergy. Cilantro has been implicated in IgE mediated hypersensitivity reactions varying from mild cases of urticaria to anaphylaxis. To our knowledge this is the first reported case of eosinophilic esophagitis secondary to cilantro. Case Description: A 31 year old man with no prior medical history initially presented to gastroenterology clinic with episodic dysphagia and food impaction for approximately 7 years. Five years prior, an EGD revealed a Schatzki ring which was treated with dilation. He was maintained on Omeprazole daily but subsequently self discontinued as he was relatively asymptomatic. The patient returned after experiencing 6 distinct episodes of dysphagia associated with increased mucous production over a 2 year period. Repeat EGD with biopsy of the lower esophagus showed "strikingly abundant eosinophils, consistent with eosinophilic esophagitis". He was started on Lansoprazole by his gastroenterologist. The patient was asked to record a food diary of all foods he consumed in relation to episodes of dysphagia. He underwent skin prick testing for corn, potato, soy, wheat, chicken, pork, egg, milk, almonds and peanuts with commercial extracts, all of which were negative. Skin prick testing to aeroallergens was positive for dust mites only. As he noted symptoms with intake of fresh salsa, the patient later underwent fresh food skin prick test to tomato and cilantro, which was positive to cilantro. ImmunoCAP testing for cilantro/coriander was negative at <0.1kU/L. Lansoprazole was changed to Omeprazole which has been shown to decrease IL-13 and eotaxin 3 levels as additional benefit. With strict cilantro avoidance measures and omeprazole the patient noted no further episodes of dysphagia at three month follow up. Conclusion: This case illustrates the importance of fresh food skin prick testing in evaluating eosinophilic esophagitis associated with intake of foods and spices. Although skin prick with commercial extracts and ImmunoCAP testing are used as first line in diagnostic work ups, fresh food skin prick testing with cilantro should be considered with a suggestive history. Rationale: Asparagus food allergy is rare and usually associated with positive skin or serum IgE testing. Methods: A 26 year-old male reported urticaria over 3 years associated with eating asparagus. He had no history of atopy. Skin testing with fresh asparagus was negative. An open challenge resulted in a diffuse urticarial rash within minutes of ingestion. Serum IgE via Immuno-CAP was less than 0.35 KU/L. Serum was analyzed using ELISA testing. Asparagus was lyophilized and protein content determined by a modified Lowry technique. The food extract was added to ELISA plate wells, incubated, washed, blocked, and re-washed. Serum was then added and the plate was incubated, washed, and anti-human IgE antibody was added. After incubation, plate was washed and pNPP AP substrate was added. Optical density readings were taken at 30 minutes and at 1,2,3, and 20 hours. Inhibition ELISA testing was also performed, with asparagus inhibited by itself. Controls included cord blood as well as serum from an atopic patient. The assay was performed in triplicate. Results: The patient's serum exhibited IgE binding to asparagus antigens at a ratio of 2.5 times that of the cord blood control. The binding was completely inhibited by preincubation of the serum with asparagus. An atopic control exhibited binding to asparagus which was also inhibited by asparagus. Immunoblot was performed and was negative. Conclusions: To our knowledge this is the first patient with asparagus allergy manifesting as systemic urticaria with negative fresh asparagus skin testing and negative commercial serum IgE testing demonstrated to have specific IgE to asparagus via an IgE-ELISA inhibition assay. Further testing is needed to identify the specific allergens involved. K.E. Bruner *1 , R. Gomez 2 , J. Freiler 2 , K. White 2 , A. White 3 , 1. Lackland AFB, TX; 2. San Antonio, TX; 3. San Diego, CA. Introduction: Pineapple is rarely reported to cause anaphylaxis. We present two cases of pineapple anaphylaxis with evidence of IgE antibodies to pineapple. Methods: Two patients with histories consistent with pineapple anaphylaxis underwent ImmunoCAP and immunoblot testing to pineapple and one underwent skin prick testing. Results: Both patients reported regularly eating pineapple prior to their initial episode of anaphylaxis. Pt 1 initially experienced a feeling of throat swelling, lip numbness and unsettled stomach after eating pineapple but did not associate it as an allergic reaction. Three weeks later, he ate 4-5 pieces of pineapple and experienced generalized sweating, prolonged abdominal cramping and recurrent vomiting that lasted approximately one hour. He had no rash or angioedema. His skin testing revealed a 15x40mm wheal and flare to fresh pineapple, 15x50mm to pineapple extract. He was skin test negative to bromelain, other fruits including fig, kiwi and papaya, and an aeroallergen panel. An immunoblot was performed which revealed a faint band at 23 kD consistent with bromelain and correlated with a weak positive Immuno-CAP of 0.42 kU/L. Patient 2 developed generalized hives, angioedema, throat closing, diarrhea and a sense of impending doom shortly after ingesting pineapple. She received steroids, epinephrine and IV fluids in the emergency department with resolution of symptoms over 90 minutes. Her initial ImmunoCAP to pineapple was 2.24 kU/L. She deferred pineapple skin prick testing due to fear of recurrent reaction. Repeat ImmunoCAP was obtained one year later and was negative at <0.35 kU/L. Immunoblot performed at that time was also negative suggesting that her sensitivity may have waned over the intervening 12 months. She was not interested in food challenge. Conclusion: Pineapple can cause anaphylaxis with presence of IgE demonstrated on skin prick testing, ImmunoCAP and immunoblot. Bromelain was identified on immunoblot as the reactive substance. One patient's ImmunoCAP converted to negative after one year indicating that sensitivity to pineapple may decrease over time. Introduction: FPIES is a non-immunoglobulin E (IgE) mediated GI food hypersensitivity which presents with profuse, repetitive vomiting, often with diarrhea leading to dehydration and lethargy after ingestion of a food allergen. The exact mechanism is unknown, however it is thought that the ingestion of an allergen causes local inflammation (mediated by T cells) leading to increased intestinal permeability and fluid shift. The most common allergens are cow's milk or soy protein, however solid food proteins can also elucidate these symptoms. The common solid food that has been known to cause FPIES is rice. Methods: An 8 month old female with a history of atopic dermatitis presented for initial consultation after she developed two episodes of profuse vomiting, listlessness and non-bloody diarrhea requiring ICU admission status post ingestion of homemade sweet potatoes. The first instance occurred at 6.5 months of age for which she was diagnosed with a viral sepsis syndrome in the setting of a positive parainfluenza culture. Symptoms then reoccurred at 7.5 months of age when she was fed sweet potato mixed with pear, and then she was diagnosed with FPIES. She had already been consuming cheerios, oatmeal, banana, peas, and broccoli. The patient's mom had questions regarding the proper timing to introduce new foods. Results: There are no specific recommendations regarding introduction of new foods for patients with FPIES. Among infants with solid food FPIES in one study, 80% reacted to more than one food and 65% were previously diagnosed with cow's milk and/or soy FPIES. Typically, if a patient is tolerating foods from one group then other foods of that group are considered OK to eat. It is suggested that in children with solid food FPIES, the introduction of grains, legumes and poultry should be delayed until the first year of life. Conclusions: It was recommended to our patients mother that she continue foods she is currently tolerating. It was also recommended that mom can introduce other fruits one at a time. Since she was eating peas, it was suggested that she try lentils as her next legume. Since she was eating oat, quinoa was recommended as her next grain. Mom was instructed to continue breast feeding. She was instructed to wait to until one year of age to consume soy and milk. Approximately two weeks later she had tolerated lentils, quinoa and blueberries at home without any issues. She still had not exposed her to dairy. Introduction: Pramoxine is a local anesthetic agent that has long been used in various surgical specialties. A few cases of contact dermatitis have been described; however anaphylaxis has not been reported. Though pramoxine has low sensitizing potential, it was implicated in our case of anaphylaxis. Case Description: A 47-year-old Caucasian man was brought to our emergency department with a widespread pruritic rash, generalized edema, chest pain, dyspnea, nausea, and diaphoresis. An acute anaphylactic reaction was suspected, and treated immediately. Serum laboratories revealed tryptase 2.6 (< 15.8 mcg/L), total IgE 44.5 (< 113 kU/L), and WBC 14.7 (4-11 x 1000/mcL) with no eosinophils. He had applied Neosporin + Pain Relief® cream (active ingredients neomycin, polymyxin B, and pramoxine) to a cut on his hand minutes prior to his anaphylactic event. He had applied topical Neosporin® ointment (without the Pain Relief component) numerous times previously without any adverse reaction. At allergy clinic follow-up 5 weeks later, topical application of Neosporin + Pain Relief® cream to the patient's forearm produced a 30 x 30-mm wheal. Skin prick testing revealed a 7 x 9-mm wheal to histamine and to pramoxine. Testing of neomycin and polymyxin B were negative. In vitro assays were performed (ImmunoCAP 1000 System, Thermo Fisher Scientific, Waltham, MA). Direct coupling of pramoxine to the solid phase was not feasible as there was no free amine or hydroxyl group available on this compound. Specific IgE against polymyxin B, neomycin, and bacitracin were not detected in the patient's serum (< 0.1 KU A /L). Therefore, of the 3 active components in Neosporin + Pain Relief® cream, pramoxine was the culprit. Discussion: Pramoxine is used only for surface anesthesia; therefore sensitization should not entail any risk of future systemic reaction. Low sensitization potential has been demonstrated, and patients sensitive to other topical anesthetics showed no sensitization to pramoxine. To our knowledge, ours is the first report describing anaphylaxis to topically applied pramoxine. Ready access to the systemic circulation seems to be a prerequisite for the development of anaphylaxis. In contemplating topical therapy, patients should consider the integrity of the skin. If the skin barrier is compromised, an anaphylactic reaction is possible. Physicians should be aware that topical pramoxine may give rise to life-threatening anaphylaxis. T. Shankar * , A. Petrov, M. Fajt, Pittsburgh, PA. Introduction: Food allergies appear to be increasing in prevalence (Boyce, JACI 2010), and aside from physical effects, lead to poor quality of life. Epidemiologic studies to date have focused on the pediatric population, and as a result, there is limited data on food allergies in adults, with available data suggesting differences in both presentation of allergy and underlying causative agents in these two groups. This study aimed to evaluate the characteristics of patients presenting to an adult Allergy/Immunology clinic with food allergy as well as to determine the most prevalent food allergies reported. Methods: We conducted a retrospective analysis of demographic and clinical data from adult patients seen in an outpatient university Allergy/Immunology Clinic from June 2010-June 2014 for a complaint of food allergy as evidenced by ICD-9 code of 995.7. Data: Preliminary results from evaluation of 10 patients demonstrated a female predominance (90%) with onset of symptoms in adulthood (age >18 yrs) in 80%. Concomitant environmental allergy was present in 80% of patients and drug allergy, most commonly penicillin, was present in 40% of patients. Though nearly all of the patients (70%) had previously sought medical care for food allergy, only 1 patient had injectable epinephrine previously prescribed at the time of visit. The most common food allergen reported and confirmed was shrimp (40%). Other confirmed food allergies were fish (salmon), peanuts and tree nuts. 80% of patients reported allergy to >3 foods, but only 30% had positive serum specific IgE testing to >3 foods. Of the 10 patients evaluated, 40% opted to have no further testing beyond the initial visit and were discharged with injectable epinephrine prescription, training for use if needed, and an action plan for food avoidance. Conclusion: Our preliminary data suggests that onset of food allergy can occur in adulthood and is under-recognized and undertreated by the medical community. Specific features such as female gender and coexistence of both environmental and drug allergies may help to identify adult patients with food allergy. Additional analysis with larger sample sizes is needed to confirm these associations. W.M. Rassbach * , J. Wang, New York, NY. As a monoclonal antibody to CD20 on B lymphocytes, rituximab is used to treat a variety of lymphoproliferative disorders, including post-transplant lymphoproliferative disorder (PTLD). It is unknown to what degree this rituximab-induced B cell depletion could affect pre-existing IgE-mediated food allergy in children. We report a case of a three year-old patient who developed hives and lip swelling to peanut butter after receiving tacrolimus immunosuppression for a living-related liver transplant performed for biliary atresia. She subsequently developed PTLD and underwent treatment with rituximab, leading to B cell depletion. In the allergy evaluation that followed, her skin prick testing revealed a negative histamine control, and her serum food-specific IgE levels were undetectable. As the patient had demonstrated clinical reactivity to peanut despite a negative serum peanut IgE, her case raises the question as to whether rituximab could have played a role in masking her IgE-mediated sensitivity to peanut and other foods. Rituximab targets actively dividing B cells, particularly those present in the peripheral blood, and as such her case may indicate that the IgE active in food allergy is being produced by B cells in a body compartment other than the peripheral blood. Background: Food allergy affects many millions of people. May develop allergy to almost any food, but in some cases it may be result of cross-reactivity between food and inhalant allergens. Clinical manifestations can be included in the pollen-food syndrome sometimes leading to trigger anaphylaxis. These syndromes are related to proteins like pathogenesis related protein family (PR-10), profilin, lipid transfer proteins (LTPs) and storage proteins. Objective: Determine the frequency of sensitization to food rich in LTPs and profilin in patients of the National Institute of Respiratory Diseases of Allergy and Immunology Department. Methods: Patients were seen at the Allergy and Immunology Department with suggestive symptoms of oral allergy syndrome or food anaphylaxis underwent questionnaire to determine functional gastrointestinal disorders with subsequent skin testing to aeroallergens and food with LTPs and profilins. Results: 42 patients were included, 75.6% were female and 26.1% male, mean of IgE levels were 506 IU / L, average eosinophils in peripheral blood 422 cells/mm 3 . 71% of patients had symptoms of oral allergy syndrome, 19% anaphylaxis to food intake and 60% had functional gastroin-testinal disorders, the most frequent was esophageal and functional dyspepsia. Positive test to aeroallergens were 76.19% Betulaceae (Birch 45.2%, Alnus 30.95%), 78.57% Oleaceae (42.5% Fraxinus, Olive 35.09%), 38.09% Asteraceae (Red Oak 38.09%), 38.9% Fagaceae (Thistle 38.09%). For food were positive test were peach, orange and plum 38.09%, raw apple, 35.7% raw broccoli, celery, 33.3% raw carrot, strawberry, 30.95% raw onion, corn, banana, kiwi, hazelnut and almond 28.57%. Positivity LTP extract 14% and 12% were positive to profilins. Conclusion: In our populations, 12% of patients were positive to profilins and 14% to LTPs. 100% was positive to multiple pollens which is similar to the prevalence reported worldwide 10-30% patients with pollen allergy react to profilin. This is of great importance since it is considered a risk factor for developing food allergies as well as most of these patients with oral allergy syndrome. Also it has been reported that anaphylaxis is more common in patients with only sensitization to LTPs. 1. Chicago, IL; 2. DeKalb, IL; 3. Toronto, ON, Canada; 4. New York, NY. Introduction: Food allergy prevalence is increasing globally in developed and developing countries especially in children. Avoidance is still the best recommendation for management; however, strict avoidance impacts quality of life. Recent research has focused on food allergen threshold levels, the lowest amount of an allergen that causes a reaction. After years of recommendations to avoid allergens at any level, it is unknown how families feel about thresholds. This would be the first international study of its kind to examine differences in food allergy types, severity, diagnosis, and opinions about food allergy thresholds between countries. Methods:A survey prepared by FARE gathered consumer comments on allergen thresholds and was distributed by patient organizations in 16 countires to 10,225 respondents. The survey was designed for people with food allergies, family members, and caregivers who purchase their food. The public was asked to comment on opinions of food allergen thresholds, current approaches to managing food allergies through food selection, and knowledge of thresholds. The results from these surveys were compiled and analyzed by country. Results: Differences were observed by country in percent of respondents with food allergy to each of the common food allergens, percent of respondents with history of severe reaction, source of diagnosis, knowledge of food allergy thresholds, and willingness to consume food that contains varying amounts of food allergen. Overall, while 73% of respondents said they had some knowledge about allergen thresholds, only 21% said they would buy a product that contained levels of an allergen low enough that it would not trigger a reaction and 4% would purchase a product with levels only capable of triggering a mild reaction. Responses to all threshold opinion questions varied by country. Conclusion: There are clear differences in food allergy in each of the countries participating in the survey. This is the first study of its kind to analyze the breakdown of food allergy by common allergen and severity in each country and opinions about the emerging topic of food allergen thresholds. It is apparent that if a food contains an allergen a family is avoiding, they may avoid the food regardless of thresholds and allergen levels. As this topic develops, patient input is crucial as industry labeling policy shifts. A. Doshi * , S. Leonard, San Diego, CA. Introduction: FPIES is a non-IgE mediated gastrointestinal food hypersensitivity that is characterized by delayed profuse, repetitive vomiting and often diarrhea. Severe cases can also lead to acute dehydration; weight loss and failure to thrive can result if chronic. We present 2 cases of FPIES caused by avocado, a trigger that has not previously been reported. Case 1: A breastfed 10-month-old male presents with a history of reflux, failure to thrive, and intermittent vomiting, which improved with restricted maternal diet. Solids were introduced at age 6 months, one new food every 3 days. At age 7 months, 4 hours after the second introduction of peas, zucchini and avocado he experienced 2 episodes of emesis and appeared lethargic, returning to baseline by the next morning. Shortly thereafter he developed repetitive vomiting, paleness, limpness and lethargy 3 hours after the second introduction of sweet potato and squash, separately. He was treated in the emergency room with ondansetron and intravenous fluids for the sweet potato episode, but recovered at home after the squash reaction. At this point he was diagnosed with sweet potato and squash FPIES. At age 10 months, avocado was ingested again, and 3 hours later he developed vomiting, limpness, paleness and lethargy. He recovered at home after 3 hours. Case 2: A 9-month-old female was diagnosed with rice and milk FPIES after presenting with a history of repetitive vomiting 2 hours after ingestion of rice and milk-based formula, separately, at age 7 months. When avocado was introduced at age 12 months, she developed repetitive episodes of vomiting 5 hours after ingesting a few bites. She was back to baseline a few hours later. Discussion: These cases highlight avocado as an unreported trigger of FPIES. The most common causative foods are milk, soy, rice, and egg; reactions to meats, nuts, and other fruits/vegetables have also been reported. To our knowledge, there have not been any other published accounts of avocado FPIES. There is often a delay in FPIES diagnosis due to the lack of biomarkers and because symptoms overlap with other more common disease processes, such as gastroenteritis. FPIES is a clinical diagnosis based on history and it is important to not overlook uncommon or unreported triggers. Introduction: Food protein-induced enterocolitis syndrome is a non-IgE mediated food allergy which usually presents in infancy as severe vomiting and diarrhea. Onset of symptoms are delayed and occur 2-4 hours after ingestion of a food. FPIES patients often experience delayed diagnosis due to the similarity of its presentation with gastroenteritis, surgical abdominal processes, and sepsis. This case illustrates that acute FPIES can be accompanied by profound dehydration and a high clinical suspicion should be maintained for the potential clinical sequelae of severe dehydration. Case Summary: An exclusively breastfed 6 week old full-term male was transferred to our institution for the management of hyponatremia and severe dehydration in the setting of one week of worsening vomiting and diarrhea. He had been breastfeeding and gaining weight appropriately until six days prior to admission. He presented with 6 days of multiple episodes of nonbloody, nonbilious emesis and four days of loose nonbloody diarrhea. He had 0.52 kg weight loss over one week's time, with exam notable for sunken fontanel, delayed capillary refill and dry mucous membranes and laboratory workup notable for hyponatremia to 128. He required rehydration with IV fluids, and during the hospitalization he developed persistent rightward eye deviation which progressed to apnea and bradycardia requiring resuscitation. Electrolytes were normal and brain MRI/MRV revealed thrombosis of the medullary vein. Hypercoagulable workup was negative. Workup of his vomiting and diarrhea was negative, including: LFTs, lipase, infectious stool studies, testing for neurometabolic disorders, anti-enterocyte and FoxP3 staining as well as normal IgA staining of intestinal biopsies. His hospital course was significant for multiple attempts to restart breastmilk or alimentum complicated by recurrence of vomiting and diarrhea; he was eventually able to tolerate NeoCate with resolution of GI symptoms and was discharged home on NeoCate. At followup visits he demonstrated normal growth pattern and stooling pattern on NeoCate, but at 28 months had accidental exposure to a small amount of milk and developed diarrhea secondary to this exposure. Although FPIES is a diagnosis of exclusion given the severity of symptoms in this patient, and the reproducibility of his symptoms, FPIES to milk seems is the most likely diagnosis. Introduction: Two methods for treating Eosinophilic Esophagitis (EoE) are off-label use of swallowed steroids and dietary therapy. For swallowed steroids, the two main options are swallowing MDI actuated inhaled corticosteroids or mixing budesonide into a slurry with sucralose (Splenda®). However, some patients do not like sucralose. We describe our treatment success with other agents used to mix budesonide. Methods: We retrospectively reviewed data from EoE patients treated with swallowed budesonide at our institution. EoE was defined using the standard consensus criteria of > 15 eosinophils/hpf (eos/hpf) on esophageal biopsy after ruling out gastroesophageal reflux. A value of < 15 eos/hpf was considered adequate improvement. IRB approval and informed consent was obtained from all subjects. Results: Fifty-four children between ages 2 and 18 years (mean 6 years) were treated with swallowed budesonide over a 5 year period and had sufficient data to review. The most common delivery vehicle for budesonide was sucralose (n=32, 59%). A variety of other agents were also used: 11 patients used apple sauce; 3-honey; 1-alternating apple sauce and ice cream; 1-banana puree; 1-pear sauce; 1-hot cocoa mix; 1-rice cereal; 1-tapioca starch; 1-xanthan gum; and 1-compounded formulation. Of the patients using sucralose, 30 of 32 patients had improvement on their esophageal biopsy (down to mean 1.27 eos/hpf). Of the 11 apple sauce patients, 7 had improvement (down to mean 2.86 eos/hpf); 2 had no repeat biopsy data, 1 did not respond adequately to budesonide, and 1 improved after switching to honey as the vehicle. The patient on alternating apple sauce and ice cream went from 60 eos/hpf to 20 eos/hpf. All 3 honey patients had improvement (down to mean 3.33 eos/hpf). The patients using banana puree, pear sauce, hot cocoa mix, xanthan gum and the compounded formulation as the vehicle all had improvement to 0 eos/hpf. The patients using rice cereal or tapioca starch did not have improvement on their biopsies. Conclusion: Splenda® (sucralose) is the most commonly used delivery vehicle for swallowed budesonide in EoE patients. Various other vehicles can also be effective, including apple sauce, pear sauce, honey, banana puree, hot cocoa mix, xanthan gum, and a compounded formulation. Certain other agents, however, may not work. Case Presentation: An 18-year-old female presented to the allergy clinic with concern for beer intolerance. She drank beer for the first time at age 14 and did not develop symptoms. Recently she has begun experiencing lip and mouth tingling and numbness within minutes of drinking beer, followed by nausea and vomiting within an hour. She denied additional skin or mucosal symptoms, and never experienced respiratory symptoms. Her symptoms occur with a single sip of beer and are present with every exposure, regardless of the type of beer. She has consumed wine, champagne and distilled spirits without any adverse reactions, both before and since her symptoms with beer. She also reports developing a localized, urticarial rash if beer is splashed on her skin. Her past medical history is remarkable for migraine headaches for which she takes sumatriptan as needed. She has no history of atopic diseases. She has no known drug allergies. Her mother has allergic rhinitis. Review of systems and physical examination were unremarkable. Skin prick test was adequate for interpretation with a histamine control of 10mm/34mm and a negative glycerin control. She reacted to barley extract (5mm/17mm), malt extract (10mm/32mm) and Pabst Blue Ribbon beer (10mm/40mm). She did not react to corn, hops, wheat or yeast (Saccharomyces cerevisiae) extracts. She was instructed to avoid beer and food or beverages containing barley or malt. Follow up was arranged to perform oral food challenges to barley and malt, in order to determine whether she can safely consume foods containing these ingredients. Conclusion: Although uncommon, beer allergy should be suspected in a patient who reacts adversely to beer and whose presentation is consistent with an allergic reaction. Several beer components have the potential to cause IgE-mediated reactions, including barley, hops and yeast, in addition to various other starches. [3] Identifying a specific allergen, although often difficult, will assist in allergen avoidance and prevent unnecessary dietary restrictions. A. Kaur 1 , G. Koch 2 , S. Durham 3 , J.M. Portnoy 4 , Z. Li 1 , J. Maloney 1 , H. Nolte *1 , 1. Whitehouse Station, NJ; 2. Chapel Hill, NC; 3. London, United Kingdom; 4. Kansas City, MO. Introduction: Number needed to treat (NNT) and needed to harm (NNH) is a quantitative approach to assess relative efficacy and safety of a treatment. Lower NNT indicates more effective treatment, and higher NNH indicates safer treatment. NNTs for allergic rhinitis (AR) treatments depend on the clinical response definition and range from 3-34.5; NNHs depend on the frequencies and types of adverse events and range from 13-167. We determined the NNT and NNH to quantify benefit-risk for 2 sublingual immunotherapy tablets (SLIT-T) approved for Timothy grass-(and related grasses) or short ragweed (RW)induced AR. Methods: For grass SLIT-T 2800 BAU (MK-7243; Merck/ALK-Abelló), efficacy data vs placebo from 3094 subjects was used for NNT; safety data from 4195 subjects was used for NNH evaluation. For RW SLIT-T 12 Amb a 1-U (MK-3641; Merck/ALK-Abelló), efficacy data vs placebo from 644 subjects was used for NNT; safety data from 1814 subjects was used for NNH evaluation. In the absence of an established responder definition, NNT was based on 2 exploratory post-hoc responder definitions. Definition 1 required the subject to achieve ≥50% well days during the pollen season (grass full season, RW peak season). Well days were days with no rescue medication use, and with the worst score as none or mild for each of the 6 symptoms measured. Missing data were considered non-well days. Definition 2 required the subject's total combined symptom and medication score (TCS) to be ≤3 (~10% of the observed endpoint range to be considered a responder). NNH was based on the adverse event rate for epinephrine use and treatment-related systemic allergic reactions, the safety endpoints of clinical concern. IRB approval and informed consent was obtained from all subjects. Results: For grass SLIT-T, the NNT was 7.9 using the ≥50% well days definition and 9.4 using the TCS ≤3 threshold. NNH was 305 based on epinephrine use and 303 based on systemic allergic reactions. For RW SLIT-T the NNT was 10.3 using the ≥50% well days definition and 16.4 using the TCS ≤3 threshold. NNH was 230 based on epinephrine use and no harmful effect could be determined based on systemic allergic reactions. Additional sensitivity analysis corroborated the NNT results. Conclusions: Grass SLIT-T and RW SLIT-T have favorable benefit-risk ratios based on NNT and NNH. Introduction: This study investigated the percentage of patients who achieved their pre-set dust mite immunotherapy maintenance goal within 1 year. Patient demographics were analyzed to see if specific populations were less likely to achieve goal. Methods: An IRB approved retrospective chart review of all current immunotherapy patients at one allergy clinic was conducted. Only patients on standardized dust mite mix immunotherapy for at least 1 year were included. One year was chosen as the inclusion time because it would take 32-43 weekly incremental dosage steps to achieve maintenance goal if there were no gaps or skipped doses, assuming a starting dose of 0.05cc of 0.001AU-0.1AU and a goal maintenance dose of 0.20cc of 10,000AU using our standard protocol for dose advancement. Patients were excluded if they received combination shots with other allergens in the same extract vial or were on house dust immunotherapy. Chi-square analysis was used to determine if differences were significant. Results: There were 36 females and 46 males that met criteria for the study. Of the 82 patients, 41 (50%) achieved their pre-set maintenance goal target in 1 year. 41.7% of females achieved goal maintenance compared to 56.5% of males. However, chi-square analysis found no significant gender difference in achieving goal (p=0.182). Participants were also stratified into 3 different age groups: young (0-39 years), n=35, middle aged (40-59 years), n=32, and elderly (60+ years), n=15. The age group differences were statistically significant (p=0.003). Odds ratio showed middle aged patients were 3.2 times and elderly patients 6.0 times more likely to achieve goal when compared to young patients. Also, patients with no reactions to immunotherapy were significantly more likely to achieve maintenance goal than those who had reactions (p=0.027). Patients who consistently visited clinic for immunotherapy treatments were more likely to achieve goal than those who missed appointments (p≤0.000). Conclusion: Patients who consistently visit clinic for immunotherapy treatments, have no major reactions to treatments, and are older are more likely to achieve maintenance goal in a timely manner. Patients younger than age 40 are at high risk of not meeting goal. Improving success for this population may indicate a need for more resources for them such as special adherence counseling on each injection visit and visit reminders. 1. Whitehouse Station, NJ; 2. Pittsburgh, PA; 3. Minneapolis, MN; 4. Fort Worth, TX. Introduction: Saliva may carry concentrated allergen extract to areas of the gastrointestinal (GI) tract distal to the mouth and throat when swallowed after administration of sublingual immunotherapy tablet (SLIT-T) or liquid. Unabsorbed contents of a rapidly dissolving SLIT-T may be swallowed fewer times than that of a SLIT-T that takes minutes to dissolve or liquid extracts, minimizing allergen exposure contact with the non-mouth/throat GI tract. Therefore, it is important to characterize the non-mouth/throat GI adverse events (AEs) associated with each SLIT product. We report the GI AEs associated with 2 SLIT-T products approved for the treatment of Timothy grass (and crossreactive grasses) or short ragweed (RW)-induced allergic rhinitis that dissolve within seconds under the tongue. Methods: AE data from 8 randomized, double-blind, placebo-controlled trials (24-week to 1-year) of daily Timothy grass SLIT-T 2800 BAU (MK-7243; Merck/ALK-Abelló) were combined into adult and pediatric safety pools. AE data from 4 randomized, double-blind, placebocontrolled trials (two 52-week trials and two 28-day trials) of daily RW SLIT-T 12 Amb a 1-U (MK-3641; Merck/ALK-Abelló) were combined into a 28day safety pool. Results: A total of 3173 subjects received grass or RW SLIT-T and 2836 subjects received placebo (Table) . The majority of treatment-related GI AEs were associated with the oral mucosa (Table) . Dysphagia and dyspepsia did not affect a large number of subjects but did occur at a numerically higher rate than in the placebo group for each pool (Table) . Abdominal pain and gastroesophageal reflux disease (GERD) occurred in ≤1% of subjects receiving grass or RW SLIT-T. Only 1/2116 (0.05%) subjects receiving grass SLIT-T (none for RW SLIT-T) reported eosinophilic esophagitis; this AE was mild in intensity, did not result in study discontinuation, and was not assessed as treatment-related by the investigator. No serious GI AEs were reported in any subject receiving grass or RW SLIT-T. Only 2/2116 (0.09%) subjects receiving grass and 1/1057 (0.09%) receiving RW SLIT-T discontinued due to treatment-related abdominal pain or GERD. Conclusions: GI AEs associated with grass SLIT-T and RW SLIT-T treatment predominately affected the oral mucosa. Non-mouth/throat GI AEs were infrequent and rarely resulted in study discontinuation. (Figure 1) , were studied. Immunologic assessment of all patients revealed normal humoral and cell-mediated immune function. The warts had failed to respond to multiple courses of destructive modes of treatment, including cryotherapy, salicylic acid 6%, imiquimod 5%, and electrodessication These patients were started on immunotherapy consisting of intralesional injections of 0.4 mL of a Candida albicans antigen preparation (Hollister-Stier) with a concentration of 1,000 PNU/mL. This treatment was given at 3-week intervals with an average of 3.5 sessions. Results: The patients' plantar warts achieved complete and striking resolution without scarring (Figure 2a, 2b) following immunotherapy with multiple intralesional injections of Candida albicans antigen. No relapses have been reported to date (average follow-up ~2.3 years). The mechanism of action is still uncertain, but most likely due to the stimulation of a T-helper type 1 (Th1) cell cytokine response leading to induction of local and distal cell-mediated immune responses. The most common side effect was pain during intralesional injections. Conclusion: The striking favorable response to multiple stratified intralesional immunotherapeutic injections of Candida albicans antigen in these 4 patients with recalcitrant warts unresponsive to usual destructive forms of treatment, provides support for a promising effective and safe mode of immunomodulator therapy. Introduction: Controversy exists about the efficacy of self-administered SCIT protocols. We previously reported the safety study results of the United Allergy Service (UAS) self-administered SCIT protocol (WAO J 2014, 7 (Suppl 1), 24). We now report the results of the efficacy trial. Methods: An IRB/HAC approval and informed consent was obtained from all research subjects. A retrospective efficacy study was completed for 56 control patients and 60 SCIT patients (in accordance with the UAS protocol). Enrollment criteria in part included only patients: who demonstrated positive SPTs to at least 3 of 6 common study site geographic allergens (Timothy, Bermuda, Cedar, Oak, Ragweed, and Cocklebur) and had associated seasonal symptomology. The study contrasted statistical assessment of pretreatment and ongoing symptom scores (SS), medication scores ( Background: Subcutaneous specific allergic immunotherapy (SSAI), in conjunction with drug therapy and patient education, is the cornerstone for the treatment of allergic diseases. So far SSAI is the only treatment able to modify the allergic disease in addition to helping prevent the development of asthma. Complications of SSAI are rare but possible. Currently there is insufficient information to describe the incidence of adverse reactions in daily clinical practice. Collecting information that assesses SSAI's safety could help us to identify allergens, dose, time of administration, and other factors in order to reduce the incidence of potentially serious reactions. Objective: To assess the safety of SSAI in patients with allergic rhinitis from 2005-2014. Methods: Patients with Allergic rhinitis who received SSAI between 2005-2014 were included. SSAI was performed using Alk-Abello® allergens with monthly applications according to Mexican guidelines for immunotherapy. An adverse reaction to SSAI (AR) was considered as the presence of any clinical manifestation proposed by the European Academy of Allergy and Clinical Immunology. Frequency analysis and survival curve were performed using the software SPSS v. 21. Results: 1,923 patients were included, 3.7% (72) had AR, the average age of the patients was 19 years, 63% were female and 37% male, 67%(48) were children and 33% (24) adults. The most frequent allergens detected by skin test and reported as managed with SSAI were Dpt (94%) and Ash (25%). Of the 72 patients with AR, 66% were polisensitized (POL) and 34% monosensitized (MONO); 39% received SSAI with standardized allergens (E), 25% with non-standard () and 26% a combination. The average duration of the SSAI treatment was 15 months, and the AR was presented during the build up phase at 1:1 concentration in E and 1:200000 in . POL patients developed AR earlier than MONO patients, and the same phenomenon was observed when comparing vs E. Of those with AR 65% of the cases had mild reactions, 24% moderate and 11% severe. Conclusion: The polisensitized pediatric patients who received SSAI with non-standardized allergen had mild adverse reactions that were presented earlier in our population. R.K. Zeldin *1 , Y. Amistani 1 , L. Paolozzi 1 , U. Wahn 2 , 1. Antony, France; 2. Berlin, Germany. Background: The efficacy and safety of the 5-grass pollen sublingual tablet has been demonstrated in patients with grass pollen-induced allergic rhinoconjunctivitis. Using pooled data from across the development program, we evaluated the safety by age group. Methods: Patients (age: 5-65 years) with medically confirmed grass pollen-induced allergic rhinoconjunctivitis for at least 2 years were included in one of eight double-blind, placebo-controlled studies conducted worldwide. IRB/EC approval and informed consent was obtained from all patients. Those who received 300IR (9,000 BAU) 5-grass pollen sublingual tablet or placebo were considered for the age subgroup (i.e., children/adolescents [5-17 years] and adults [≥18 years]) analysis. Adverse events were monitored throughout the studies. Treatment-emergent adverse events (TEAEs) were analyzed descriptively. Results: 1,878 adults (300IR=1,038; placebo=840) and 312 children/adolescents (300IR=154; placebo=158) were included in the analysis. TEAEs were reported by 78% of adults and 84% of children/adolescents receiving active treatment and 66% and 79% of those receiving placebo. The most commonly reported TEAEs by both actively-treated subgroups were application site reactions (e.g., oral pruritus, throat irritation, mouth edema), with similar incidences. The incidence of severe TEAEs was similar in actively-treated adults (102 patients [9.8%]) and children/adolescents (17 patients [11.0%]). Similar percentages of adults and children/adolescents receiving the 300IR sublingual tablets withdrew from the studies due to TEAEs (49 [4.7%] adults and 7 [4.5%] children/adolescents), mainly as a result of application site reactions. Three serious TEAEs (hypersensitivity, angioedema and diarrhea) considered related to the study drug by the investigator were reported in adults; none were reported in children/adolescents. No activelytreated patients received epinephrine and there were no ICU admissions. Con-clusions: The incidence, nature and severity of adverse reactions reported during treatment with the 300IR (9,000 BAU) 5-grass pollen sublingual tablet were similar in the adult and pediatric populations. D.B. Golden *1 , K. Abiteboul 2 , R.K. Zeldin 2 , 1. Baltimore, MD; 2. Antony, France. Background: The efficacy of the 5-grass pollen sublingual tablet has been demonstrated in both mono-and poly-sensitized patients with grass polleninduced allergic rhinoconjunctivitis. Using pooled data from across the development program, we report the safety profile according to the sensitization status of patients at study entry. Methods: Eight double-blind, placebo-controlled studies were conducted worldwide. Patients (age 5-65 years) with medically confirmed grass pollen-induced allergic rhinoconjunctivitis for at least 2 years were enrolled. IRB/EC approval and informed consent was obtained from all patients. At entry, all patients underwent skin testing to 5-grass mix or timothy and a panel of geographically relevant aeroallergens. Mono-sensitized patients had a positive test to 5-grass mix/timothy alone and poly-sensitized ones had a positive test to 5-grass/timothy and at least one other aeroallergen. Of note, these other sensitizations were not to cause potential confounding symptoms of allergy during the period of evaluation. Adverse events were monitored throughout the study. Treatment-emergent adverse events (TEAEs) were analyzed descriptively. Results: A total of 951 patients were mono-sensitized (active=587; placebo=364) and 1,560 were poly-sensitized (active=927; placebo=633). Similar incidences of TEAEs were reported in active and placebo groups, whether patients were mono-sensitized (77.2% vs. 67.9%) or poly-sensitized (76.7% vs. 70.9%) and the most frequently reported TEAEs (i.e., application site reactions such as oral pruritus, throat irritation, mouth edema) were reported at similar incidences in actively-treated mono-and poly-sensitized patients. Mono-and poly-sensitized patients receiving active treatment reported serious TEAEs with similar frequencies (1.5% and 1.4%, respectively). Activelytreated patients withdrew due to TEAEs with similar frequencies whether monosensitized (31 patients [5.3%]) or poly-sensitized (46 patients [5.0%]). Conclusions: Treatment with the 5-grass pollen sublingual tablet was similarly well tolerated in mono-sensitized and poly-sensitized patients. Rationale: Allergic rhinitis is frequently associated with ocular symptoms which can have a significant impact on quality of life and daily activity. The effect of house dust mite (HDM) sublingual immunotherapy tablet (SLIT-T) on ocular symptoms is unknown; therefore, the efficacy on ocular symptoms of MK-8237 (Merck/ALK-Abelló), a HDM SLIT-T currently under clinical development, was evaluated in an environmental exposure chamber trial. Methods: Adults with HDM-induced allergic rhinitis with/without conjunctivitis and/or asthma (n=124) received daily MK-8237 12 DU, 6 DU, or placebo for 24 weeks in this randomized, placebo-controlled, double-blind, single-site trial. HDM exposure challenges of 6 hours duration were conducted at screening and weeks 8, 16, and 24. The total ocular symptom score (TOSS) was assessed and was the sum of 2 scores: gritty/red/itchy eyes and watery eyes, scored on a 0 to 3 point scale (maximum=6). Total nasal symptom score (TNSS) was also assessed and was the sum of scores for runny nose, blocked nose, sneezing, and itchy nose, scored on a 0 to 3 point scale (maximum=12). IEC approval and informed consent was obtained from all subjects. Results: Overall 83% of subjects reported ocular symptoms at the screening chamber challenge. Improvements of 34. 1% Background: The efficacy and safety of a sublingual tablet of house dust mite (HDM) allergen extracts was tested in an environmental exposure chamber (EEC) study, in which patients were exposed to a stable concentration of HDM allergens. Here, we focus on asthma-related symptoms. Methods: Adults (18-55 years) with medically confirmed HDM-associated allergic rhinitis were randomized in this double-blind study to receive either placebo or active treatment at the doses of 500IR, 300IR or 100IR with daily administration for 6 months. Patients with intermittent asthma controlled with therapies consistent with GINA treatment step 1 could be enrolled. IRB approval and informed consent was obtained from all patients. During the study, patients participated in five 4-hour allergen challenges in the environmental exposure chamber. Analyses were performed to evaluate the incidence of bronchospasm, asthma and asthma-related symptoms, such as dyspnea, cough, wheezing, occurring in relation to the EEC challenges (i.e., peri-EEC periods defined as the day of and the day after an allergen challenge) and that occurring outside of these periods. Results: 355 patients received at least 1 dose of the investigational product and comprised the safety set (500IR=93; 300IR=86; 100IR=89; placebo=87); 12.7% had asthma. During the peri-EEC periods, the incidence of bronchospasm, asthma and asthma-related symptoms was similar across treatment groups (range: 36.6%-44.9%). The incidence of these symptoms outside of the peri-EEC periods was approximately half of that during these periods (range: 16.1%-21.3%). The majority of patients experiencing these events did not have asthma at study entry. Seven patients in the 3 active treatment groups (none with asthma at baseline) withdrew due to peri-EEC asthma symptoms. No Patients withdrew outside of these periods. All events were of mild or moderate intensity; 2 resolved without treatment, 4 with inhaled β 2 -agonist, and 1 with an OTC treatment. Conclusions: Treatment with sublingual tablet of house dust mite allergen extracts tested in an environmental exposure chamber was generally well tolerated and without adverse effects on respiratory function. During the challenges, asthma-related symptoms of mild-moderate severity were reported in patients receiving placebo or active treatment. Traditionally, immunotherapy consisted of regular (weekly/bi-weekly) injections over several months until a maintenance stage was reached. Rush immunotherapy (RIT) allows the patient to achieve a maintenance stage much quicker than traditional immunotherapy. The purpose of the current study was to identify whether patients were more or less adherent to treatment when they received RIT. A systematic chart review was conducted on all new patients who started immunotherapy in 2011. Variables of interest included demographic factors (age, gender, etc.), potential control variables (systemic reactions, prick test results, and asthma diagnosis), RIT (hours), and duration of treatment (months). Overall, 199 patients began allergy immunotherapy at the clinic in 2011. The average patient was 33 years old, female (65.3%), and Caucasian (81.9%). The majority of patients had systemic reactions during their initial screening (92.4%) and was also prick test positive (96.0%). The patients were almost evenly divided by asthma diagnosis (yes=59.1%; no=40.9%), but analyses showed no significant differences between these two groups in regards to RIT hours and treatment duration. Patients were classified into one of three groups at the time of chart review -(1) still on treatment, (2) stopped treatment without restart, and (3) stopped treatment with restart(s). Of the 199 patients who started treatment in 2011, 97 (48.7%) were still receiving treatment with an average therapy duration of 14 months. Eighty patients (42.2%) had stopped treatment and had not restarted while 18 patients (9.0%) had stopped treatment and subsequently restarted, some on several occasions. The average number of RIT hours for patients still on treatment (M=13.37) was significantly higher than for patients who had stopped treatment (M=8.20) even if they restarted (M=2.64) [F(2, 196) = 15.57, p = .000]. Further analyses were conducted classifying patients into groups dependent upon the number of RIT hours they received, if any. Patients receiving 1-10 RIT hours had significantly lower treatment duration (M=8.3) than patients receiving no RIT (M=14.4), 11-20 RIT hours (17.2), or more than 20 RIT hours (18.77) [F(3,195) = 14.17, p = .000]. While RIT hours do play a role in treatment duration, results of these analyses suggest that another variable, possibly related to commitment to treatment, may also be involved in the prediction of adherence. Background: The World Health Organization estimates that 40% of the world population suffers any type of allergic disease lilke allergic rhinitis (AR), currently the mainstay of treatment of AR is the use of topical steroids, however the specific allergy immunotherapy (SAIT) is the only treatment capable of modifying the natural course of the disease. Currently the studies that evaluate the SAIT in patients with AR describes a favorable clinical efficacy, however the are performed in monosensitized patients, the results of these investigations have traspolated to polysensitized, without having assessed the same clinical consistency and biological support. Objective: To evaluate the efficacy of subcutaneous immunotherapy in patients polysensitized (POL) versus monosensitized (MONO) with allergic rhinitis and asthma. Methods: We enrolled asthmatic patients with AR; skin prick test were developed with ALKbello allergens classifying the groups of study in POL if there are two or more allegens positives in the test and MONO if only has a one. SAIT was developed according Mexican guideline for immunotherapy. All participants realized spirometry, Asthma Control Test, control of AR symptoms with statement of ARIA in each phase of build up and measure of eosinophils, levels of Ig E and inhaled steroids dosage at the begging and end of this phase. Statics was developed using Wilcoxon test compared 0 time vs 4 time in each grupo and U Mann Whitney between the groups with SPSSv21. Results: 23 patients participated, were xx female and xx male, the average of age was xx years old, 12 patients integrated monosensitized group and 11 polysensitized, the principal allergen was Dpt in both groups, the average of allergens in POL group were 5; FEV1 increases in each group (p = 0.006), there were decrease of 32 % in the dosage of inhaled steroid (p = 0.001) and increased of ACT questionnaire score (p = 0.001),there were not differences in these variable when comparing POL vs MONO. The levels of IgE,eosinophils and evaluation of symptoms of AR were similar in 0 vs 4 time in each and both groups. Conclusions: Immunotherapy has the same efficacy in polysensitized patients as in monosensitized Background: Subcutaneous specific allergic immunotherapy (SSAI), in conjunction with drug therapy and patient education, is the cornerstone for the treatment of allergic diseases. So far SSAI is the only treatment able to modify the allergic disease in addition to helping prevent the development of asthma. Complications of SSAI are rare but possible. Currently there is insufficient information to describe the incidence of adverse reactions in daily clinical practice. Collecting information that assesses SSAI's safety could help us to identify allergens, dose, time of administration, and other factors in order to reduce the incidence of potentially serious reactions. Objective: To assess the safety of SSAI in patients with allergic rhinitis from 2005-2014. Methods: Patients with Allergic rhinitis who received SSAI between 2005-2014 were included. SSAI was performed using Alk-Abello® allergens with monthly applications according to Mexican guidelines for immunotherapy. An adverse reaction to SSAI (AR) was considered as the presence of any clinical manifestation pro-posed by the European Academy of Allergy and Clinical Immunology. Frequency analysis and survival curve were performed using the software SPSS v. 21. Results: 1,923 patients were included, 3.7% (72) had AR, the average age of the patients was 19 years, 63% were female and 37% male, 67%(48) were children and 33% (24) adults. The most frequent allergens detected by skin test and reported as managed with SSAI were Dpt (94%) and Ash (25%). Of the 72 patients with AR, 66% were polisensitized (POL) and 34% monosensitized (MONO); 39% received SSAI with standardized allergens (E), 25% with non-standard () and 26% a combination. The average duration of the SSAI treatment was 15 months, and the AR was presented during the build up phase at 1:1 concentration in E and 1:200000 in . POL patients developed AR earlier than MONO patients, and the same phenomenon was observed when comparing vs E. Of those with AR 65% of the cases had mild reactions, 24% moderate and 11% severe. Conclusion: The polisensitized pediatric patients who received SSAI with non-standardized allergen had mild adverse reactions that were presented earlier in our population. Rationale: Gastroesophageal reflux disease (GERD) is a chronic disease and very common in the evaluation of chronic cough. Oftentimes, GERD is silent and patients do not realize they are experiencing symptoms. When treated, the dosing of medication is often a significant component in ensuring improvement and efficacy in therapy. Yet, under-dosing seems to be a common problem that is not meeting the needs of appropriate therapy. Methods: 28 patients with GERD (diagnosed by EGD or classic symptoms) were evaluated in clinic. The patients had failed conventional proton-pump inhibitor therapy with omeprazole 20mg once daily. Patients were randomized to two groups. 14 patients were placed on omeprazole 20mg twice daily; the remaining 14 patients were placed on omeprazole 40mg once daily. 12 weeks of therapy were performed and the REFLUX-QUAL (RQS) quality of life (QoL) questionnaire was used to evaluate symptom improvement. Results: The REFLUX-QUAL (RQS) questionnaire is a validated and simple tool consisting of 8 items covering the principal domains of the patients' QoL. A score is rapidly obtained ranging from 0 to 100; the higher the score, the better the QoL. The median score amongst both groups at Week 0 was 36. Patients in the Omeprazole 20mg twice daily group had RQS scores improve by an average of 16 points at Week 12 whereas the 40mg once daily group RQS scores improved by an average of 44 points at Week 12, an almost three-fold improvement. Conclusions: GERD is a common diagnostic consideration in the Allergy and Asthma practice. It has been my finding that increased frequency (qday vs. BID) of failed omeprazole doses do not alleviate or improve symptoms. Rather, it appears that an increased dose of omeprazole is associated with much improved REFLUX-QUAL symptoms scores and quality of life (QoL). A steady state of the same failed dose is not appropriate to control symptoms. It is likely that this might be a class finding and relatable to other proton pump inhibitors. Background: There is a paucity of data examining patterns for inpatient pediatric Allergy and Immunology (A/I) consultations. Objective: This analysis was to determine the reasons for inpatient consultation to the A/I service at a tertiary care pediatric referral center. Patient demographics and patterns for establishing A/I follow-up (if recommended) were also examined. Methods: We performed a retrospective chart review of all inpatient A/I consults from January 1, 2013 to December 31, 2013. Results: In 2013, 82 inpatient consults were placed to the A/I service, with 76 unique patients. Of these, 55 represented patients who had not previously been evaluated by the Section of Allergy and Immunology. Fifty-six percent (31/55) of new patient consults were to assess for possible immunodeficiency, 16% (9/55) for angioedema/anaphylaxis, 13% (7/55) for possible food allergy, 7% (4/55) for adverse drug reactions, 5% (3/55) to assist with immunoglobulin replacement therapy, and 2% (1/55) for rash. Of the consultations placed for possible immunodeficiency, 71% (22/31) were for patients under 2 years of age. The majority of new inpatient consults were requested by Infectious Disease (ID) (23%, 13/55), Hospital Pediatrics (14%, 8/55), and Gastroenterology (13%, 7/55). The remaining 50% of consults were placed by 11 other subspecialty services. Outpatient A/I follow up was recommended for 31 (56%) of the 55 new patients evaluated in the hospital. Of these 31 patients, 55% (17/31) presented to our clinic for scheduled follow up evaluation. Established patients of the A/I service accounted for 27 inpatient consults on 22 unique patients. Fifty-two percent (14/27) of these consults were for co-management related to a previously diagnosed immunodeficiency. The majority (67%, 18/27) of consults for established A/I patients were placed by ID (20%, 11/55) and Hospital Pediatrics (13%, 7/55). Conclusions: Our review of inpatient A/I consultations demonstrates the need for expert evaluation of both allergic and immunologic disorders in the inpatient setting. Additionally, inpatient evaluation by the A/I service is helpful for establishing outpatient A/I follow-up for patients new to the A/I service. Understanding the inpatient consultation patterns has implications for A/I fellow training, resident and medical student education, as well as patient care. Introduction: Many clinicians under-utilize and incorrectly dose epinephrine in anaphylaxis. Residents report being confident in diagnosing and managing anaphylaxis despite limited clinical experience with this relatively uncommon condition. We designed a simple training module to teach interns how to use an EpiPen®, as well as developed and validated a scoring tool to reliably measure an intern's autoinjector proficiency. Methods: We developed the EpiPen® proficiency assessment tool (E-PAT), a two-part tool including a checklist that outlines 8 steps of proper autoinjector use and questions about a clinician's prior EpiPen® experience. We recruited 58 interns from an ACGMEaccredited internal medicine training program at an academic medical center during orientation where they underwent skills training. This study was IRB approved. Each intern participated in a 30-minute training module, followed by an assessment. First, the interns watched a part of the manufacturer's instructional video. Second, an allergist recapped the key points in the video and encouraged questions. Third, the interns used EpiPen® trainers to practice on themselves and their colleagues, with the opportunity to receive feedback from the observing allergist. Finally, two independent coders (an allergist and a senior resident with allergy training) used the E-PAT to individually assess each intern's EpiPen® proficiency. Results: All 58 interns participated in the study. Prior to this activity, 74% of the interns reported having never used an EpiPen® trainer, none have used an EpiPen® in the past year and 7% have ever used an EpiPen®. Over one-third (37%) of the interns knew someone who uses an epinephrine autoinjector. After the training, the interns' mean EpiPen® proficiency score was 7.5±0.85 out of a maximum of 8 points (range 6-8), suggesting that this module was effective. Inter-rater reliability for each item on E-PAT was high and the overall kappa was 0.63, p<0.0001. Conclusion: Interns reported limited experience with epinephrine autoinjectors prior to starting residency. Our brief training module resulted in high, measurable EpiPen® proficiency immediately. E-PAT demonstrated substantial inter-rater reliability. Future studies will assess whether residents retain proficiency longitudinally and if E-PAT is equally reliable when used by non-clinician coders. Background: Several treatment options exist for the treatment of hereditary angioedema (HAE), a rare genetic disease that manifests in episodes of swelling in different parts of the body. Attenuated androgens, including danazol, are well established for long-term use but are associated with multiple side effects and contraindications. C1 esterase inhibitor concentrate (C1-INH) replaces the missing or dysfunctional protein and is recommended as a firstline treatment by international guidelines. Case Description: A 51-year-old male patient with HAE had his first symptoms of HAE (abdominal pain) at an age of 13 years. He was initially misdiagnosed with indigestion and allergies. His first laryngeal attack occurred at an age of 23 years. Upon diagnosis of HAE, the patient received a daily dose of 200 mg danazol for long-term prophylaxis. During danazol therapy, he experienced side effects, including increased transaminases, and arterial hypertonia. Reduction to a daily dose of 100 mg danazol triggered HAE attacks (≥ 1 attack per week). When the patient expressed the wish to switch from danazol to C1-INH, he was trained in intravenous self-infusion and learned how to recognize early symptoms of HAE attacks. Danazol was then reduced stepwise (tapering down over several weeks). During this period, recurrent attacks occurred approximately every 8 days and were successfully treated with self-infusion of human pasteurized nanofiltered C1-INH (pnf-C1-INH, Berinert ® ; CSL Behring, Marburg, Germany). Currently, the patient experiences HAE attacks every 5 days. All attacks are treated successfully with self-infusion of pnfC1-INH. The patient has no side effects and transaminases have returned to normal. Arterial hypertonia is ongoing but is well-controlled with lercanidipine. Conclusions: Self-infusion of pnfC1-INH is an effective and safe alternative for patients with severe HAE who experience side effects or show lack of response to attenuated androgens. Before switching from long-term prophylaxis with danazol to acute treatment with pnfC1-INH, we inform the patient about a possible increase of attack frequency and train the patient in intravenous self-administration techniques. We do not stop androgens immediately and taper down danazol over several weeks. G. Steven * , Milwaukee, WI. Objectives: Health decision-makers involved with treatment, coverage and payment policies are increasingly developing policies that seek information on "real-world" (RW) data base outcomes. Motivated by these initiatives, we have begun to develop a framework to assist health-care decision-makers in dealing with RW clinical data, especially related to treatment, coverage and payment decisions. Methods: Since 2008, a relational database has been developed and refined to complement the EHR by providing clinical decision support in ways that are not currently possible in most certified software. Data from skin testing, spirometry, eNO analysis, impulse oscillometry are combined with medication lists, immunotherapy formularies and injection administration records, smoking history, demographics, quality of life assessments and immunization status. Results: We defined RW clinical information (P4P) as data used for decision-making in day-to-day clinical care that are more practical than the low-level paradigms formulated by data collected in conventional randomized controlled trials (RCTs) "evidence-based medicine." We considered several characterizations: by type of outcome (clinical, economic, and patient-reported), by hierarchies of evidence (which rank evidence according to the strength of research design), and by type of data source (supplementary data collection alongside RCTs, large simple trials, patient registries, administrative claims database, surveys, and medical records). P4P data base key issues: 1) the importance of RW data; 2) limitations of RW data; 3) data depends on the circumstance; 4) good practices for collecting and reporting RW data; 5)good process in using RW data in coverage and reimbursement decisions; 6) the need to consider costs and benefits of data collection; 7) need for modeling; and 8) the need for continued stakeholder dialogue. Conclusions: Realworld databases are essential for sound coverage and reimbursement decisions. Of greatest utility is our use of clinical data to provide high-level treatment decisions that maximize outcomes. It is critical that policymakers recognize the benefits, limitations, and methodological challenges in using RW data from private practice data bases (P4P), and the need to consider carefully the costs and benefits. Background: Medication errors are a very common problem, which can lead to a range of sequela, including disclosure with no harm to a substantial degree of morbidity. As a result, medication reconciliation is being implemented as a formal process in many hospitals. Similarly, inaccurate medication allergy lists can lead to increased cost to the system, unnecessary repeat allergy testing, inappropriate or inferior antibiotics being prescribed on readmission or even allergic reactions. We hypothesized that the majority of inpatients tested for penicillin allergy were not allergic and that this information was not adequately documented in the EMR or communicated to the general practitioner. Methods: We retrospectively reviewed the charts of all inpatients at a teaching hospital that were seen by a consultant allergist over the year of 2012. Data col-lected included basic demographics, penicillin allergy test results, current allergy status on the EMR, readmission rates, prescribed antibiotics and discharge summary contents. Results: 146 patients were tested for penicillin allergy in 2012. Of these, 144 (98.6%) were not allergic to penicillin. Although orders were written in the charts of 145 (99.3%) patients to update their allergy status after testing, 32 (22.23%) of these patients with negative tests were still listed as allergic to penicillin in the EMR. Only 19 (15.2%) discharge summaries notified family physicians of the allergy testing results and discharge summaries were missing for 25 (20%) patients. Further assessment of 50% of the charts revealed that in 41% of cases the negative allergy test resulted in a change of antibiotics to penicillin or its derivative. Of the 60 readmitted patients, 20 (33%) were still listed as allergic to penicillin in the EMR (only one patient tested positive on skin testing) and 14 (70%) of the 20 patients required antibiotics. 12 of the 14 patients (86%) were prescribed antibiotics in the penicillin family despite their positive allergy status. Conclusions: A significant proportion of health records were not amended following antibiotic allergy testing and the patient's new allergy status was not communicated to the majority of general practitioners in the discharge summary. A more efficient and reliable system needs to be implemented to ensure changes to allergy status are communicated to all members of the healthcare team. G. Steven * , Greenfield, WI. Clinical and demographic data have been entered in a diagnosis-related administrative database (P4P) at AASC since 2008. This database was used to estimate the prevalence of ragweed seasonal allergic rhinitis and to examine the capture of data in P4P, considered in different time frames. Methods: A case-finding algorithm identified patients with a positive skin test to ragweed among those who had had skin testing done since 1996 and healthcare contact at AASC within the last six years for whom we had current and correct contact information. Prevalence rates were calculated as the ratio of the number of identified patients to the total number of allergic rhinitis patients tested. Results: Of the 6877 skin test records in the database, 534 patients were identified who had at least a moderate SPT to short ragweed (approximately an 8mm wheal or greater), had a diagnosis of allergic rhinitis, were not already on SCIT, had a current email address on file and, based on the prescribing information of the recently approved sublingual ragweed extract tablet, were between the ages of 18 and 65. Only 42 of these patients called in to request more information about this new treatment option. Since this medication had been only recently approved, we found lack of formulary coverage to be a significant obstacle to getting patients treated with this new therapy. Nonetheless, although there was only about 2 weeks' time between the drug appearing in pharmacies and the last day to start therapy this year (12 weeks prior to the start of the RW season, per the package insert), we were able to initiate treatment with 12 patients. Conclusions: Clinical and administrative healthcare databases in private practice today can be important tools in identifying populations that may benefit from new therapies, notifying patients of new information (e.g., boxed warnings) regarding medications they are currently taking, as well as identify potentially eligible patients for clinical research trials as part of the drug development process. However, data collection needs to be judicious, relevant and used on a daily basis in order to have a positive impact on clinical care. Background: Allergic diseases affect 40% of the population with an increasing prevalence. In the SIDRIA study, prevalence of allergic diseases changed with the age: asthma and rhinitis raised (respectively from 9.3% to 10.3% and 12.3% to 20.9%), while eczema decreased (from 15.9% to 11.9%). However, data on younger children are lacking. The aim of the present study is to assess the prevalence of allergic and respiratory diseases as well as related risk factors in a population of 3 to 5 years old preschool children. Methods: The study includes children attending 4 Nursery Schools in an urban district of Rome. Data on the allergic diseases were collected using standardized questionnaire (SIDRIA-2) and Skin prick tests (SPT) was performed. We investigated the association of atopy, rhinitis, wheezing and asthma with a list of potential deter-minants, calculating the Odds Ratios (OR) and 95% Confidence Limits (95%CI) from logistic regression models. Results: 494 children (46% male) aged 3-6 years were studied. More than 75% were breastfed (> 2 months) and 87% were weaned after the 5 th month of life. Most of them started attending school after the 11 th months and 64% had previously attended daycare. Sixty-seven percent of children had at least 1 sibling; 72.3% lived at home with at least other 3 persons; 42% reported to live in a street with heavy traffic; 20.6% had a pet at home, and 22.9% had smoking parent. SPT was positive in 12% of children. Asthma was positively associated with nationality not Italian (OR: 2.54, 95%CI: 1.09-5.92), breastfeeding >1 month (OR: 2.78, 95%CI: 0.97-7.97), daycare attendance (OR: 2.26, 95%CI: 1.14-4.52), mother's atopy (OR: 2.04, 95%CI: 1.13-3.66), and dermatitis (OR: 2.02, 95%CI: 1.13-3.61). Allergic rhinitis was positively associated with father's atopy (OR: 3.11, 95%CI: 1.30-7.44), and dermatitis (OR: 11.6, 95%CI: 4.52-29.6). Atopic status was positively associated with mother's atopy (OR: 2.09, 95%CI: 1.15-3.77). Conclusion: Our study found a high prevalence of atopic sensitization in children under five years, and confirms the high prevalence of allergic diseases, to an extent comparable to those seen later in life. The mother's atopy increases the risk of developing asthma, father's atopy increases the risk of allergic rhinitis while pollutants (smoke and vehicular traffic) are not significant risk factors in these setting. Introduction: Breastfeeding and vaginal delivery are known to be beneficial to a child's health. This study investigates the prevalence of atopic disorders in children who are breastfed versus bottle fed and method of delivery, spontaneous vaginal delivery (SVD) or cesarean section (C-section). Methods: A retrospective chart review was conducted on 100 patients 0.5 to 17 years of age (average 6.3 years of age ± 4.3). Patients were separated by duration of breastfeeding or formula fed with no breastfeeding. Breastfed infants and toddlers were subdivided into 5 groups based on length of feeding, <3 months, 3-<6 months, 6-<9 months, 9-<12 months, and 12 or more months. Prevalence of atopic skin disorders, such as eczema and atopic dermatitis (AD), Immunoglobulin E (IgE) disorders, such as food allergies, asthma, allergic rhinitis (AR), and a family history of pertinent atopic diseases (FHX) were identified in each patient. Student's t test was performed. Results: Out of the 100 children, 52 were males and 48 were females. The children breastfed for 9-<12 months and born via SVD group had the lowest atopic skin disorders, but had a low FHX. The 6-<9 months breastfed and born via C-section group had the lowest amount of IgE disorders, but the highest amount of atopic skin disorders. The 6-<9 months breastfed SVD group had the highest FHX. The 12+ months breastfed SVD group had the highest number of IgE disorders. All breastfed children had a lower occurrence of atopic skin disorders with a p value of 0.02. With regards to breast fed versus formula fed, the 9-<12 months breastfed group had the lowest atopic skin disorders, IgE disorders, and FHX prevalence. The 6-<9 months breastfed group had the highest atopic skin disorders and FHX. The <3 months breastfed group had the highest occurrence of IgE disorders. Conclusion: Breastfeeding for 9-<12 months seemed to reduce the amount of atopic skin disorders and IgE disorders; however, the genetic lack of prevalence of atopy may explain this observation. Children breastfed for <3 months had higher incidence of atopy. Patients that were born via SVD were shown to have a less likely occurrence of atopic skin disorders and FHX and a higher occurrence of an IgE disorder when compared to patients born via C-section. A future study with a larger population size is needed to confirm these results. Rationale: TMJ, or temporo-mandibular joint disease, is a common disorder with significant morbidity affecting the quality of life for many people in the United States. TMJ is more common in non-hispanic Caucasian women and has been attributed to pain threshold, behavioral factors, and past trauma. However, to date no clear etiology has been identified and this disease is likely multifactorial. Characterized primarily by pain, headache, and ear pain, TMJ is very difficult to treat given the complex nerve root anatomy of the skull and foramina. Given the significant morbidity TMJ carries on patients, we hypoth-esize a correlation between the presence of TMJ symptoms and the Epworth sleep score which measures degree of daytime drowsiness during a variety of activities. Methods: After informed consent and IRB approval, 44 subjects were identified to have doctor diagnosed TMJ consisting of a constellation of tenderness to palpation over the TMJ with joint movement. 763 subjects did not have clinically diagnosed TMJ in a university academic allergy/immunology clinic. Results: The average Epworth Sleepiness Scale (ESS) score amongst subjects with TMJ was 7.8 in comparison to controls with an average ESS score of 7.1. Conclusions: Our results show a positive correlation between the presence of TMJ symptoms and Epworth sleep score. Our findings indicate a need to further evaluate the relationship between TMJ symptoms, day time sleepiness, and the role of sleep hygiene in symptomatic TMJ patients to improve patient quality of life. Q. Meng *1 , S. Nagarajan 1 , L. Bielory 2 , 1. Piscataway, NJ; 2. Springfield, NJ. Background: The prevalence of allergy skin test (IST), ocular and nasal symptoms (ONS), and asthma has been reported from N-II and N-III. However, the associations of among these symptoms over time are unknown. Methods: N-II (1976 -1980 and N-III (1988 N-III ( -1994 collected allergy IST data, ONS and asthma prevalence. IST, ONS and asthma results were assessed for 6 common allergens (Ags) to N-II and N-III: cat, ragweed, perennial rye, oak, Bermuda grass, and A. alternata. The IST+ (sensitized for 1 or more Ags) populations were then assessed for ONS, and asthma status. ONS was identified using survey responses and asthmatics based on having a history and still having asthma. The associations among IST, ONS and asthma were characterized with contingency tables and correlation analyses. Results: IST+, ONS, and asthma status were collected from N-II (n=11,044) and N-III (n=10,833). The number of IST+(n=2144), ONS(n=991), and asthma(n=275) from N-II; and IST+(n=4538), ONS(n=3242), and asthma(n=547) from N-III. Comparing N-II to N-III, the prevalence increased: asthma 2.0 times, IST+ 2.2 times, ONS 3.3 times. The concurrent prevalence of asthma, ONS, and +IST increased 5.3 times (all p< 0.0001; Χ 2 test). From N-II to N-III, sensitivity increased to an increasing number of Ags. ONS prevalence in N-III was 2-3 times > N-II (p< 0.0001, except for all 6 Ags, p=0.069). Within each NHANES survey, the associations among +IST, ONS and asthma were all statistically significant with stronger associations observed in N-III vs. N-II. Correlation coefficients between asthma and positive skin tests doubled or tripled in N-III (r=0.04 in N-II & 0.12 in N-III for oak). A linear association (p< 0.0001) between the cumulative number of IST+ (ranging from 0-6) and the prevalence of ONS or asthma was stronger in N-III. Conclusion: From N-II to N-III, the prevalence of asthma, ONS, and IST increased with greatest associated with more than one symptom. From N-II to N-III, sensitivity increased to an increasing number of Ags. The associations among IST, ONS, and asthma became stronger during N-III vs. N-II. Supported in part by US EPA STAR Grant #: RD834547; Observational, Laboratory, and Modeling Studies of the Impacts of Climate Change on Allergic Airway Disease; PI: Leonard Bielory, M.D. M. Hogan 1 , A. Wonnaparhown 2 , R. Scherr *2 , N. Wilson 2 , 1. Reno, NV; 2. Las Vegas, NV. Introduction: Eosinophilic esophagitis (EE) is an inflammatory esophagitis which can be associated with IgE sensitization to food and aeroallergens. Food avoidance, swallowed glucocorticosteriods, and proton pump inhibitors have been utilized therapeutically in pediatric EE patients. Information regarding pediatric follow-up of EE is limited. Methods: Patients (1-18 years) with biopsy proven EE co-managed with pediatric GI from 2010-2013 in the UNSOM A/I clinic and having at least one follow-up visit were evaluated retrospectively for skin test IgE sensitization to food, aeroallergens, and EE symptoms. Statistics were performed with Fischer's exact test with graphpad.com. IRB exclusion was obtained. Results: 23 children with EE meeting study criteria were identified. IgE sensitization occurred to food in 87%, aeroallergens 96% and 87% of children had sensitization to both. A/I and GI therapy for EE and AR increased after the first A/I visit with 9/23 swallowing GCS, 3/23 on proton pump inhibitors, 8/23 avoiding most likely contributing foods and 16/23 with prescribed aeroallergen avoidance or immunotherapy for allergic rhinitis or/asthma. Most common food sensitizations identified were legumes (14/23) and tree nuts (8/20). Other foods were noted at lesser frequencies. Common aeroallergens identified in the population in order of frequency were cat 69%, sagebrush 63%, maple 61%, dog 57% and timothy 54% of the study population. Eosinophilic esophagitis symptoms of stomachache (p<0.037) and GERD/heartburn (p<0.033) improved with this comprehensive care. Conclusion: Pediatric EE is a complex inflammatory disease with multiple possible triggers and follow-up information after initial evaluation is limited. Aeroallergens and food IgE sensitization was notable in this population. Comprehensive care in this population resulted in decreased GI symptoms of pain and should be studied further as a working model to improve pediatric EE care. Introduction: National training programs answer to the Accreditation Council for Graduate Medical Education (ACGME) to meet competency requirements. Allergy and Immunology (AI) requirements include core competency achievements in allergy (hypersensitivity). Methods that empower learners to take ownership in their own course curriculum are not standard, yet can maximize content retention important for accreditation, exam scores and practice performance. Our objective was to develop an innovative and effective Hypersensitivity Course (HSC) by/for AI fellows. Methods: Needs assessment was performed for actual competence and perceived efficacy in aeroallergen diagnostics, allergic disorders and procedures, based on in-training exam results, patient/procedure logs and confidence/experience/satisfaction. Gaps in allergy knowledge, practice and self-efficacy were identified and fellows became partners in creating an allergy-focused and shared-group interactive course with expert oversight. Modified flipped design with project-based learning and reallife applications to master allergy was applied. Results: AI fellows successfully designed (2008-2009) and implemented (2009-2014) their own HSC course to master ACGME AI competencies in allergic diseases/procedures, aeroallergen identification and desensitizations, and continue to modulate it (by practice parameters/literature updates and learner needs) and teach it (weekly-10 months/year). All HSC AI fellows (2008-2014) reached increased in-training exam scores and board pass rates (100%), met log requirements to 50%ile (2013-2014), ranked it highest among courses, achieved increased allergy selfefficacy and, in 2013, earned the ACGME David Leach Award for a traineedriven educational program. Conclusion: HSC AI fellows conquered comprehension-performance in allergic diseases and procedures with confidence and competence through becoming teachers while learners. They became empowered as leaders in their own sustainable curriculum for themselves and their clinical team. Their HSC methods may serve as a feasible and effective model not only for meeting AI training requirements but also for those seeking mastery in allergy practice and knowledge. K. Kulich 1 , B. Tiplady *2 , D. Banerji 3 , 1. Basel, Switzerland; 2. Peterborough, United Kingdom; 3. East Hanover, NJ Introduction: Patient-reported outcomes are increasingly important in evaluating the severity and impact of chronic conditions such as chronic obstructive pulmonary disease (COPD). Electronic patient diaries (eDiaries) have been found to be highly acceptable to patients, who often prefer them to paper. Also, eDiaries allow time-stamping of entries, automatic tailoring of entries to the time of day, and limit entries to valid options. Methods: Qualitative patient interviews have been used in the development of a new COPD eDiary, which includes five symptom items and two impact items rating bother and difficulty (BD). Two morning assessments (since awakening, M1; since last assessment, M2) and one evening assessment (E) were made each day. The eDiary was completed by a subset of 209 patients (140 males, aged 44 -86) in a 26week QVA149 SHINE study. Results: Patient compliance with the eDiary was 90.1% over the 7-day baseline period, and 80.0% at Week 26. The frequencies of reporting of each of the 5 symptoms were ≥60% of baseline, with shortness of breath, mucus/phlegm and cough occurring in ≥70% entries while chest tightness and wheezing occurring in ≥60% of entries over the 26-week study period. Exploratory factor analysis gave a single factor, including both symptoms and the BD items. Internal consistency was extremely high, being >0.95 for all three assessment points (M1, M2 and E) at baseline, Week 12 and Week 26. Thus the scale is unidimensional, so that the overall mean of the seven items (OM7) could be used as a summary measure. Test-retest reliability was assessed in 44 patients who showed no change in their symptoms between baseline and Week 1. The intraclass correlation coefficients (ICC) for OM7 were 0.97 for M1 and M2 and 0.96 for E. The lower 95% confidence limit was >0.92 in all cases. The eDiary was sensitive to change: the effect size for change over 26 weeks in patients identified as having improved on both clinical and patient global measures, was large (0.79) as compared to a minimal change in patients classified as uncertain/no change (0.16). Anchor-based comparison suggests that a change of 0.6 on the OM7 Score of the eDiary (range 0-10) represented a meaningful change for patients. Conclusion: The new COPD eDiary showed good measurement properties, being valid, reliable, internally consistent, and sensitive to change. Eosinophilic esophagitis (EoE) is a chronic, immune/food antigen-mediated disease characterized clinically by esophageal dysfunction and histologically by eosinophil-predominant inflammation with overexpression of cytokines, including interleukin 13 (IL-13). RPC4046 (previously known as ABT-308) is a humanized, high-affinity, neutralizing antibody that prevents IL-13 binding to both IL-13Rα1 and IL-13Rα2. The first-in-human (FIH) study (NCT00986037) was a single center, randomized, double-blind, placebo-controlled, 3-part, dose escalation study. RPC4046 was administered to healthy volunteers (HV) and patients with mild to moderate, controlled asthma to assess the safety, tolerability and PK of single escalating intravenous (IV) doses or multiple escalating subcutaneous (SC) doses. Twenty HV and 15 asthma patients received single IV infusions of RPC4046 (0.3 to 10 mg/kg) or placebo. Subsequently, 12 asthma patients received 3 weekly SC doses of RPC4046 (0.3 or 3.0 mg/kg) or placebo. IRB approval and informed consent was obtained from all research subjects. RPC4046 pharmacokinetic (PK) parameters were similar after a single IV infusion between HV and asthma patients. Following multiple SC administration, T max was ~4 days, exposure was dose-proportional and the absolute bioavailability was ~70%. In 27.8% of subjects receiving RPC4046, measurable anti-drug antibodies were detected transiently, which did not appear to affect PK or safety. The AE profile was similar in HV and asthma patients and with different administration routes. For RPC4046 subjects, all AEs were mild to moderate in severity, with none reported as probably related to study drug. There were no dose-related increases in AEs and no discontinuations due to AEs. The proportion of subjects reporting an upper respiratory tract infection was greater among those receiving RPC4046 compared to placebo. A single SAE of worsening foot bunion was reported in Group 1 HV and considered not related to study drug by the investigator. There were no study drug related infusion-related reactions and no suggestion of worsening asthma. These results support further investigation of RPC4046 for allergic/inflammatory diseases involving IL-13, including the ongoing phase 2 clinical trial in adults with EoE (NCT02098473). PK Parameters (Mean ± SD) of RPC4046 Following a Single IV Infusion in Healthy and Asthma Subjects a N = 4 for Cmax and Cmax/Dose for Groups 2 and 3 b Mean ± SD c Harmonic mean ± pseudo-standard deviation Previously, we have shown the efficacy of Omalizumab, a recombinant, humanized, chimeric anti-IgE monoclonal antibody, for the treatment of Severe Chronic Idiopathic Urticaria (CIU) patients not controlled on oral antihistamines. Clinical observations have led us to believe that sulfasalazine, an antiinflammatory agent, composed of a sulfapyridine covalently linked to 5-aminosalicylic acid, may provide additional efficacy in sub optimally controlled Chronic Idiopathic Urticaria (CIU) patients that remain symptomatic on an H1-Receptor Antagonist, Cetirizine. In this open labeled 16 week trial, 60 adult patients with symptomatic Severe Chronic Idiopathic Urticaria using Cetirizine, 10 mg orally QD, were randomized to continue the existing therapy Cetirizine or to add on Sulfasalazine 500 mg oral tablets (increased by 500mg weekly) until control of clinical symptoms occurred or until reaching a maintenance dose according to patient's weight, 30 to 50 mg/kg per day, in 2 evenly divided doses, not to exceed 2g per day to the existing therapy Cetirizine. The end points of the trial include: weekly itch severity score on a scale. The mean of measurements at the end of the 16-week trial revealed a significant improvement in all parameters examined in the treatment group receiving Sulfasalazine (as addon to the existing therapy), compared to the other group. In conclusion, the addition of Sulfasalazine to the combination of Cetirizine is more effective than Cetirizine, for the treatment of Severe Chronic Idiopathic Urticaria patients. It appears that when Sulfasalazine is added to the combination H1-receptor antagonist, Cetirizine, the end points and symptom scores are significantly improved. R. Furcha 1 , A. Amin *2 , 1. Nyon, Switzerland; 2. Parsippany, NJ. Introduction: An over-the-counter (OTC) cough and cold syrup containing paracetamol, phenylephrine, and guiafenesin was reformulated with flavour agent 316282, which has been shown to cause a warming sensation in the mouth and back of the throat. This agent is included for the taste of the product, but also may have a soothing effect appropriate to the indication. The ability of a warming sensation to help patients suffering from cough, cold, and flu feel better is well known anecdotally. However, there are no studies to date that have evaluated the warming sensation of liquids, rubs, or drug ingredients in terms of patient acceptability and preference. Objective: We sought to assess the warming sensation associated with the flavour agent 316282 in terms of patient acceptability. Methods: This was a single dose, single cohort, single treatment arm, open-label study. Subjects received one 30 mL dose of syrup containing paracetamol (500 mg), phenylephrine (10 mg), guaifenesin (200 mg) and flavour agent 316282 (0.15% weight/volume). Onset and duration of the warming sensation in the mouth/throat were recorded, and subjective assessments of strength and appeal of the sensation and overall acceptability of the product as a cough and cold remedy were surveyed. IRB/HAC approval and informed consent was obtained from all research subjects (or parents for adolescents). Results: Of 51 subjects included, 47 (92.1%) experienced a warming sensation within 60 seconds of ingestion. The median duration of the sensation was 100 seconds (95% Confidence Interval: 82, 112 seconds). Most subjects (71%) liked the warming sensation; no subjects expressed significant dislike (i.e., very much or extreme dislike) of the sensation. About 67% thought the strength of warming was "just about right." There were no safety concerns, and the syrup was well tolerated and effective for treating cough and cold symptoms. Conclusions: The flavour agent 316282 is associated with a warming sensation, which may be a desirable attribute for a cough and cold product for some patients. Lack of a control group -i.e., unflavored syrup -limits interpretation of the findings. More data are needed to overcome the challenge of appropriate controls for these types of studies. MO; 2. New York, NY; 3. Stockton, CA; 4. Bellevue, NE; 5. Ingelheim am Rhein, Germany; 6. Groningen, Netherlands. Background: In many patients (pts) current asthma control is inadequate, resulting in impaired quality of life and increased risk of exacerbations. T helper 2 (T H 2) immune response mediates allergic airway inflammation, may influence individual asthma expression, and is the target of many asthma treatments. Once-daily tiotropium Respimat ® add-on to at least medium-dose inhaled corticosteroid (ICS) ± long-acting β 2 -agonist (LABA) maintenance therapy provides sustained lung function improvements in pts with symptomatic asthma. Pre-specified analyses of pooled data from replicate Phase III, randomized, double-blind, placebo-controlled, parallel-group trials were performed to assess whether tiotropium Respimat ® treatment response was dependent on T H 2 inflammatory status. Methods: Pts with severe symptomatic asthma (PrimoTinAasthma ® , n=912) from two 48-week trials of tiotropium Respimat ® 5 mg addon to high-dose ICS (≥800 mg budesonide or equivalent) + LABA, and pts with moderate symptomatic asthma (MezzoTinA-asthma ® , n=2100) from two 24week trials of tiotropium Respimat ® 5 mg or 2.5 mg or salmeterol add-on to medium-dose ICS (400-800 mg budesonide or equivalent), were evaluated. Peak and trough forced expiratory volume in 1 second (FEV 1 ) were assessed (pooled data) in the T H 2-low and T H 2-high subgroups according to baseline total serum IgE ≤ or >430 mg/L, blood eosinophils ≤ or >0.6×10 9 /L, and clinical judgment of allergic asthma as "no" or "yes". COPD was excluded. Results: 3012 pts were included in the treated set, with baseline demographics and disease characteristics balanced between treatment regimens and subgroups. Peak and trough FEV 1 responses were improved with tiotropium Respimat ® versus placebo, independent of total serum IgE level, blood eosinophil count, and clinical judgment of allergic asthma (Table) . Safety and tolerability of tiotropium Respimat ® were balanced compared with placebo. Conclusion: Once-daily tiotropium Respimat ® add-on to at least medium-dose ICS ± LABA maintenance therapy provides sustained improvements in lung function, independent of T H 2 inflammatory status, in pts with moderate or severe symptomatic asthma. Tiotropium Respimat ® add-on therapy may therefore be beneficial in both allergic and nonallergic asthma. Adjusted mean difference for tiotropium Respimat® versus placebo at Week 24 (mL) aValues for active and placebo groups combined. Pts may be included in more than one subanalysis; bFor treatment × subgroup interaction BID, twice-daily; QD, once-daily DX-2930 is a fully human monoclonal antibody inhibitor of plasma kallikrein (pKal) under investigation for prophylaxis against acute attacks of hereditary angioedema (HAE). It is well established that pKal plays a critical role in HAE pathogenesis but the minimum level of pKal inhibition necessary to prevent attacks is unknown. We hypothesize that continuously maintaining levels of pKal inhibition associated with peak plasma drug levels (80 nM) of ecallantide (approved in the US to treat acute HAE attacks) would prevent attacks (mid-dose hypothesis). Alternatively, lower or higher levels may be necessary. To inform future clinical trial design we performed analyses to evaluate these low, mid, and high-dose hypotheses. Publicly available data from OPuS-1, a clinical study of the pKal inhibitor BCX-4161 for HAE prophylaxis (BioCryst Pharmaceuticals Inc.), was reviewed. In vitro inhibition of pKal by ecallantide and DX-2930 was assessed using a synthetic substrate assay. Modeling was conducted using pharmacokinetic (PK) data from a single ascending dose study of subcutaneous DX-2930 in healthy subjects. IRB approval and informed consent was obtained from all subjects. Review of the OPuS-1 data suggested that therapeutic response correlated with higher plasma drug concentrations of BCX-4161 and that pKal inhibitor levels below 80 nM might offer only partial prophylactic effect, lending further credence to the mid-and high-dose hypotheses over the low-dose hypothesis. In an in vitro pKal assay, DX-2930 and ecallantide displayed comparable pharmacodynamic activity at 80 nM concentrations. Thus, continually maintaining DX-2930 drug levels above 80 nM should attain the level of pKal inhibition delineated by the middose hypothesis. PK modeling predicts that chronic DX-2930 dosing will yield steady state plasma drug concentrations above 80 nM with 100 mg every 2 weeks (or 300 mg every 4 weeks) and above 200 nM with 300 mg every 2 weeks. Available data support the mid-and high-dose hypotheses for long-term prophylaxis of HAE with DX-2930. Chronic dosing with 100 mg and 300 mg is estimated to provide therapeutic coverage relevant to testing the mid and high-dose hypotheses in an efficacy and safety study. Plans for future clinical trial efforts will further be refined by PK and biomarker data from an ongoing Phase 1b study in HAE subjects. J. Maik *1 , L. Bielory 2 , 1. New York, NY; 2. Springfield. Introduction: Histamine receptor antagonists bind to several types of receptors causing various wanted and unwanted physiological responses. By identifying the affinity levels (Ki) of ligands to three Histamine receptors (H1, H2, H3), one could utilize a given antagonist by identifying low Ki values to unwanted ligand-receptor binding (H3 which is consider to be an itch agonist), and high Ki values for wanted ligand-receptor binding (H1, H2) Background: Hereditary angioedema (HAE) due to deficiency in C1 inhibitor is characterized by recurrent attacks of tissue swelling affecting multiple anatomic regions. Recombinant human C1 esterase inhibitor (rhC1INH) was previously shown effective for treatment of angioedema attacks in patients with HAE by reducing the time to symptom relief. In addition to onset of relief, the duration of attack symptoms is recognized as a clinically meaningful endpoint. The current analysis reviews the time to reach minimal symptoms after treatment with rhC1INH across six clinical studies. Methods: HAE patients treated with rhC1INH for acute angioedema attacks in three randomized-controlled trials (RCT) and three open-label (OL) studies were reviewed. Patients treated in the RCTs received rhC1INH or placebo (saline) for a single angioedema attack. In the OL studies, patients could be treated with rhC1INH multiple times for repeated attacks. Severity of attack symptoms at each affected anatomical location was assessed using a 100 mm visual analog scale (VAS) prior to treatment and at regular intervals following administration of study medication. Time to minimal symptoms was defined as occurring when VAS scores were <20 mm at all attack locations. Data were analyzed by study. IRB approval and written informed consent was obtained from all patients. Results: In the three RCTs, a total of 145 patients were randomized to receive either rhC1INH at doses of 50-100 IU/kg or saline. The median time to minimal symptoms for patients in rhC1INH 50 IU/kg groups (240, 247 min) and 100 IU/kg groups (245, 480 min) was significantly shorter than for patients in the saline groups (362, 1101, 1440 min), p<0.05 for all within study comparisons. In the OL studies patients were treated with an initial dose of 2100 IU or 50 IU/kg with an option for a second dose. Data were available on 171 patients who received rhC1INH for 449 acute attacks. Across the three studies, the median time to minimal symptoms was 241 minutes for the 2100 IU dose group, and 240 and 243 minutes for the 50 IU/kg groups. Conclusions: In addition to providing rapid onset of relief, as previously shown, treatment with rhC1INH resulted in consistently short duration of attack symptoms in HAE patients with acute angioedema attacks across six clinical studies. Rationale: Hereditary Angioedema (HAE) is a rare autosomal dominant disease that is caused by a deficiency in C1, a serine protease inhibitor. It is currently estimated that the prevalence of HAE worldwide is 1:50000. There are several drugs that have been developed to prevent and/or treat HAE. We report on most of these drugs in order to highlight the challenges of treating HAE in some parts of the world and to provide awareness of this condition, especially to help assess travel risks for patients with diagnosed HAE and to advocate for patients in areas without sufficient HAE drugs. Methods: The study was conducted by literature reviews, case reports and by obtaining relevant information from the manufacturers. Results: We identified the main categories of drugs that are in current use in the treatment of hereditary angioedema. The therapies used to treat HAE focus: on either replacing the deficient or dysfunctional C1 protein; or, on blocking the activation of specific pro-inflammatory proteins. These include: recombinant human C1 inhibitors (INH), bradykinin antagonists, and kallekrein inhibitors. Bradykinin B2 antagonists were widely available in most of Europe and in the US. Plasma kallekrein inhibitors are only available in Israel and in the US. Plasma derived C1-INH products are available in the US for HAE prophylaxis; but are approved for both propylaxis and treatment in eleven European countries. The most widely available drug was a human C1-esterase inhibitor; which was found in the US, Europe, parts of South America and Asia. A recombinant human C1 inhibitor is available in all 27 European Union countries, plus Norway, Iceland and Liechtenstein. Conclusions: Overall, there is a distribution differential in the availability of hereditary angioedema drugs. These drugs are mostly available in the European Union and in the United States. Most other countries outside these zones have either one or none of the therapies that are available for the prophylaxis and treatment of HAE. Patients should take precautions when traveling to countries without HAE therapies and have a plan of care available in case of an exacerbation. Also, since ill-health seems to be a trigger for HAE, patients should ensure that vaccinations and preventive medications are up to date, in addition to having their HAE medications available with them. Introduction: The objective of this study was to evaluate the safety of a new topical ocular anti-allergic formulation, Olopatadine HCl, 0.77% in subjects 2 years of age and older. Methods: This was a multicenter, double-masked, vehicle-controlled, parallel-group safety study. Asymptomatic adult and pediatric subjects were randomized in a 2:1 ratio to receive Olopatadine 0.77% or vehicle once daily in both eyes for 6 weeks. Safety assessments were conducted at Day 0, weeks 1, 3 and 6. Safety assessments and parameters included adverse events (AEs), best-corrected visual acuity (BCVA), ocular signs, intraocular pressure (IOP), dilated fundus exam and vital signs (blood pressure, heart rate). Results: The safety population included a total of 499 subjects ranging from 2 to 74 years of age; 330 subjects were in the Olopatadine 0.77% group and 169 subjects were in the vehicle group. Treatment-related adverse events occurring in ≥ 1% subjects included blurred vision (4.5% vs 4.1%), abnormal sensation in eye (2.1% vs 4.1%), dry eye (2.4% vs 3%), eye irritation (0.3% vs 3%), and dysgeusia (2.4% vs 0%), in olopatadine and vehicle groups, respectively. No deaths or serious adverse events were reported during the study. No subjects with exposure to Olopatadine 0.77% discontinued study participation due to an adverse event. Based upon analysis and review of safety parameters measured over the course of this trial, no clinically meaningful differences were noted between the treatment groups. Conclusion: Olopatadine 0.77% was welltolerated in the overall safety population and no safety concerns were observed with the higher concentration of Olopatadine. Background: The goal of asthma treatment is to achieve control and minimize future risk. Here we assess the efficacy of once-daily tiotropium Respimat ® add-on to inhaled corticosteroid (ICS) ± long-acting β 2 -agonist (LABA) maintenance therapy in patients with symptomatic asthma: lung function, asthma control, and asthma worsening data are presented. Methods: Four Phase III, randomized, double-blind, placebo-controlled, parallel-group trials were evaluated: two 48-week trials of tiotropium Respimat ® 5 mg add-on to high-dose ICS (≥800 mg budesonide or equivalent) + LABA (PrimoTinA-asthma ® ) in patients with severe symptomatic asthma; two 24-week trials of tiotropium Respimat ® 5 mg or 2.5 mg or salmeterol add-on to medium-dose ICS (400-800 mg budesonide or equivalent) in patients with moderate symptomatic asthma (MezzoTinA-asthma ® ). Lung function was assessed as peak forced expiratory volume in 1 second (FEV 1 ) within 3 hours post-dosing (0-3h) and trough FEV 1 . Asthma symptoms and control were assessed as responder rate (percentage of patients with minimally important change of ≥0.5) using the seven-question Asthma Control Questionnaire (ACQ-7). Time to first episode of asthma worsening (pre-specified as exacerbation) was also assessed. Results: Tiotropium Respimat ® provided statistically significant and sustained improvements in both peak FEV 1(0-3h) and trough FEV 1 . A higher proportion of patients achieved an ACQ-7 response with tiotropium Respimat ® compared with placebo (Table) . Risk of asthma worsening was reduced with tiotropium Respimat ® compared with placebo: 31% risk reduction [RR] with 5 mg (hazard ratio [HR] 0.69; 95% confidence interval [CI] 0.58, 0.82; p<0.001) in PrimoTinA-asthma ® at Week 48; 13% RR with 5 mg (HR 0.87; 95% CI 0.69, 1.08; p=0.211) and 34% RR with 2.5 mg (HR 0.66; 95% CI 0.52, 0.84; p<0.001) in MezzoTinA-asthma ® at Week 24. Safety and tolerability of tiotropium Respimat ® were balanced compared with placebo. Conclusion: Once-daily tiotropium Respimat ® add-on to at least medium-dose ICS ± LABA provided significant and sustained improvements in lung function, improved asthma control, and reduced risk of asthma worsening in adults with moderate or severe symptomatic asthma. ACQ-7 response with tiotropium Respimat® compared with placebo gic rhinitis (PAR).This is a double-blind, placebo controlled, cross-over study. Fifteen subjects with symptomatic PAR (Total Nasal Symptom Score, TNSS≥5 out of 12) received VitD (4,000 I.U./day) and placebo each for 6 weeks. TNSS (reflective and in the previous 3 weeks) and Rhinitis Related Quality of Life Questionnaire (RQLQ) were evaluated at 5 study visits (baseline, and 3, 6, 9, and 12 weeks later). This study was performed in the period November 1, 2013 to March 20, 2014, to try to avoid seasonal fluctuations of serum VitD due to sun exposure. All study subjects signed an informed consent and this study was approved by the Main Line Hospitals Institutional Review Board. Data were analyzed by the two-tail t test for unpaired data and the Pearson correlation coefficient. The study subjects' average age was 77.5±7.3 years, the Body Mass Index (BMI) was 26.5±4.9, the duration of rhinitis was 57.9±17.8 years, the total medication score (TMS) was 1.9±1.1. Baseline serum vitamin D was 29.7±10.6 I.U. and serum calcium was 9.6±0.5 mg/dl. TNSS was 7.3±2.2 (reflective) and 8±2.6 (3 week). RQLQ score was 58.3±30.9. After the 6 week VitD supplementation, its serum levels increased significantly (to 39.7±8.9 I.U., p<0.01), whereas serum calcium remained unchanged. Reflective TNSS (4.6±2.7 and 4.3±3.4 for placebo and VitD at 3 weeks, respectively), 3 week TNSS, and RQLQ (including its individual values) remained unchanged compared to placebo (p>0.4). There was no association between serum VitD and age, BMI, duration of rhinitis, or TMS (p≥0.57). However, in subjects with poorly controlled symptoms (TNSS ≥8 of 12, RQLQ for nasal symptoms ≥16 of 24), there was an inverse association with serum vitamin D (r>-0.55) that was significant for the 3 week TNSS (r=-0.81, p=0.008, n=8). In summary, VitD supplementation for 6 weeks did not improve nasal symptoms or RQLQ in elderly subjects with PAR. These results and the inverse association between VitD and nasal symptoms will require confirmation in larger studies. Introduction: Intranasal antihistamines or steroids must be delivered in a volume and with a technique that allows optimal deposition and distribution within the nasal cavity. Too little spray volume may not cover the entire nasal cavity, whereas excess volume may result in outflow from the front of the nasal cavity or backflow into the throat. In the case of Dymista (AZ/FP) Nasal Spray, it has been suggested that two generic sprays of the components is equivalent to the single AZ/FP product, although no evidence exists to support that approach. This in vitro evaluation simulated nasal deposition and distribution of AZ/FP compared to sequential sprays of the commercially available AZ and FP components. Methods: A model of a normal adult human nasal cavity was used to visualize deposition and distribution of nasal spray products. A single spray of Dymista (0.137 mL [137 mg of azelastine/50 mg of fluticasone propionate]) or single sequential sprays of azelastine nasal spray (0.137 mL) followed by either Flonase or generic fluticasone propionate nasal spray (0.100 mL) were manually actuated into the model. The interior of the cast was coated with a water-sensitive dye that changes color when exposed to aqueous-based formulations. A slight vacuum (15 L/min) was applied during spray delivery to simulate inhalation. Results were photographed using anterior and lateral views. Results: Three replicates of Dymista showed no dripping or back flow from the nasal cavity. Visually, the turbinates in the anterior third of the nasal cavity were coated with nasal spray. In contrast, three replicates of azelastine nasal spray followed in 1 minute by either Flonase or generic fluticasone nasal spray showed significant dripping from the nostril and toward the back of the nasal cavity. Visually, the turbinates were coated with nasal spray as was the top portion of the nasopharynx. Conclusions: A single spray of Dymista resulted in retention in the nasal cavity compared to sequential administration of the two component products, which caused dripping from the front of the nostril and run-off to the nasopharynx. The two components of AZ/FP Nasal Spray delivered sequentially provide a spray volume that exceeds the volume of the nasal cavity and therefore may negatively impact therapeutic efficacy and patient tolerability. Introduction: Astepro 0.10% and 0.15% are currently approved for use in patients 6 years and older with seasonal allergic rhinitis (SAR) or perennial allergic rhinitis (PAR). The objective of this clinical trial was to evaluate the safety and efficacy of the 0.10% and 0.15% azelastine formulations compared to placebo at a dosage of 1 spray per nostril twice daily in pediatric patients ≥6 to <12 yrs with perennial allergic rhinitis (PAR). The results of this study were the basis of approval for the pediatric indication. Methods: This was a 4-week, randomized, double-blind, parallel-group study in patients ≥6 to <12 years of age with moderate-to-severe PAR. The studies were IRB-approved and all patients or guardians signed written, informed consent before participation. The 12-hour reflective Total Nasal Symptom Score (rTNSS), consisting of nasal congestion, runny nose, sneezing and nasal itching, was the primary efficacy variable. Symptoms were scored twice daily on a 0 to 3 scale (0=none, 1=mild, 2=moderate, 3=severe) such that the maximum daily rTNSS was 24. Treatment group comparisons were made by analysis of covariance (ANCOVA). Results: A total of 489 patients were randomized to treatment and >90% of patients in each treatment group completed the study. The change from baseline in rTNSS was statistically significant compared to placebo for both formulations (0.15%; P = .005 and 0.10%; P = .015). Treatment with the 0.15% formulation resulted in a mean change of -3.45 rTNSS points from a baseline value of 16.60. The 0.10% formulation resulted in a mean change of -3.37 points from a baseline value of 16.35. The mean change with placebo was -2.48 from a baseline value of 16.09. The most frequently reported adverse events with the 0.15% formulation were epistaxis (4.3%), nasal discomfort (4.3%), dysgeusia (3.7%), and sneezing (2.5%). The most frequently reported adverse events with the 0.10% formulation were epistaxis (4.8%), dysgeusia (2.4%), URI (2.4%), and sneezing (1.8%). There were no serious or unexpected adverse events. Conclusions: Both the 0.10% and 0.15% Astepro formulations were effective and well tolerated in this pediatric study population. Introduction: Azelastine hydrochloride (AZ) with fluticasone propionate (FP) in a single nasal spray (Dymista) is approved for the treatment of patients 12 years of age and older with seasonal allergic rhinitis. The objective of this study was to evaluate the safety of AZ/FP Nasal Spray compared to FP Nasal Spray, administered as 1 spray per nostril twice daily in pediatric subjects ≥4 years to <12 years with allergic rhinitis (AR). Methods: This randomized, openlabel, 3-month study was conducted at 42 investigational sites in the US. The study was approved by a central IRB and informed consent (from caregiver) and pediatric informed assent (from subjects 7 years and older) were obtained before participation. Qualified subjects had a history of AR, were in good health, and had no evidence of nasal mucosal erosion, nasal ulceration, nasal septum perforation, or any significant nasal disease. Subjects were randomized in a 3:1 ratio to AZ/FP (n=304) and FP (n=101) and were stratified by age as follows: (1) ≥4 years to <6 years; (2) ≥6 years to <9 years; and (3) ≥9 years to <12 years. Safety was assessed by subject and/or caregiver-reported adverse events (AEs), nasal examinations, vital signs, and laboratory assessments. Results: Overall, 94% of subjects treated with AZ/FP and 92% treated with FP completed the study. Completion rates were similar in each age strata. The percentage of subjects with AEs was comparable between treatment groups and across the age strata. The most frequently reported AEs with AZ/FP and FP, respectively, were: epistaxis (10% and 9%), headache (7% and 3%), cough (4% and 3%) and pyrexia (3% and 2%). The discontinuation rate due to AEs was 2% with AZ/FP and 4% with FP. Laboratory parameters showed no meaningful increase in mean or median values in either treatment group. Improvements in nasal examination findings were observed in both treatment groups and in each age stratum and there were no findings of nasal mucosal ulcera-tion or septal perforation. The two treatment groups were comparable for mean changes in vital sign measurements regardless of age stratification. Conclusions: AZ/FP and FP were well-tolerated during this 3-month study when administered as 1 spray per nostril twice daily in pediatric subjects ≥4 years to <12 years with AR. B. Stanaland *1 , R. Settipane 2 , S. Gawchik 3 , N. Ruiz 4 , F. Hampel 5 , 1. Naples, FL; 2. Providence, RI; 3. Upland, PA; 4. Somerset, NJ; 5. New Braunfels, TX. Introduction: The efficacy of azelastine HCL (AZ) and fluticasone propionate (FP) in a single nasal spray (Dymista [AZ/FP]) for treating nasal symptoms of seasonal allergic rhinitis (SAR) has been demonstrated in four wellcontrolled clinical trials of 2 weeks duration. 1,2,3 The reflective total ocular symptom score (rTOSS), consisting of itching, watering, and redness, was a secondary variable in the studies. This post-hoc analysis compared the efficacy of AZ/FP to monotherapy with AZ, FP, and placebo for the treatment of ocular symptoms of SAR. Methods: A total of 3996 patients 12 years of age and older with moderate-to-severe SAR were included in the analyses. The studies were IRB-approved and all patients signed written, informed consent before participation. Patients were randomized to AZ/FP, AZ, FP or placebo nasal spray administered 1 spray/nostril twice daily (total daily doses: AZ=548 mg; FP=200 mg). The three ocular symptoms were scored twice daily on a 4-point scale (0=no symptoms; 1=mild, 2=moderate, 3=severe) such that the maximum daily score was 18. Treatment group comparisons of change from baseline in rTOSS were made by analysis of covariance (ANCOVA). Time to response (defined as a ≥50% change from baseline) was evaluated by log-rank test. Results: Overall, patients treated with AZ/FP had a significantly greater improvement from baseline in rTOSS compared to AZ (P=.048), FP (P<.0001), and placebo (P<.0001). Patients treated with AZ/FP achieved at least a 50% reduction in rTOSS significantly sooner than patients treated with FP (P=.02) or placebo (P≤.0001). The difference approached significance versus AZ (P=.08). When patients with baseline rTOSS ≥8 were analyzed (n=3341; representing 84% of the total population), significant differences were seen compared to AZ (P=.04) as well as to FP (P<.01) and placebo (P<.001). Conclusions: In this population of patients with moderate-to-severe SAR, AZ/FP improved ocular symptoms to a significantly greater extent than AZ or FP alone. Background and Objectives: To assess the symptom severities of allergic rhinitis patients sensitive to multiple allergens compared to single house dust mite allergen. Subjects and Method: Total 56 allergic rhinitis patients were classified into 2 groups. The multiple allergens group was defined as patients sensitive to multiple allergens including house dust mite in MAST. The single allergen group was defined as patients sensitive only to house dust mite. Symptom severity was evaluated with visual analogue scale (VAS) for following symptoms: postnasal drip, anosmia, sneezing, itching, nasal obstruction, cough, sputum, headache, and rhinorrhea. For in vitro test, total IgE and blood eosinophil count were also analysed. IRB approval and informed consent was obtained from all research subjects. Results: Among 56 patients, 32 patients were classified to the multiple allergens group and 24 patients were classified to the single allergen group. The mean symptom scores of multiple allergens group for postnasal drip, anosmia, sneezing, itching, nasal obstruction, cough, sputum, headache, rhinorrhea were 4.56±3.4, 1.25±2.40, 1.25±3.40, 4.25±3.11, 3.44±3.56, 5.75±3.30, 3.87±3.25, 3.50±3.45 and 5.21±3.17. On the other hand, the mean symptom scores of single allergen group for postnasal drip, anosmia, sneezing, itching, nasal obstruction, cough, sputum, headache, rhinorrhea were 2.75±2.77, 1.29±3.01, 1.29±3.01, 4.46±3.60, 3.41±3.67, 5.75±3.13, 2.88±3.03, 2.75±3.57 and 3.70±3.22. Total summation of symptom scores for two groups was 33.75±22.24 and 37.75±16.89 each. Statistically these scores showed no differences between two groups as in vitro test, total IgE and blood eosinophil count. Conclusion: Symptom severities of allergic rhinitis patients sensitive to multiple allergens showed no differences with those of patients sensitive only to house dust mite. S. Nsouli * , Danville, CA. Since chronic inflammation is the histopathologic landmark of otitis media with effusion, clinical observations have led us to believe that the combination of a nasal antihistamine Azelastine and corticosteroid Fluticasone propionate, with an oral antibiotic may be more effective than monotherapy with an oral antibiotic in the treatment of serous otitis media. We studied forty adult patients in a randomized open labeled 2-week trial to compare the efficacy of the combination nasal Azelastine and Fluticasone propionate (137mcg/50 mcg), one spray per nostril twice daily, with an oral antibiotic Amoxicillin/Clavulanate potassium (875mg/125mg) every 12 hours, for the treatment of otitis media with effusion. The efficacy of the treatment options was assessed using pneumatic otoscopy, impedance tympanometry, and audiometry to monitor the clinical course of the middle ear effusion in both treatment groups. In the combination group nasal Azelastine and Fluticasone with oral antibiotic, a resolution of otitis media with effusion occurred at the 8 th day. In contrast in the group treated with monotherapy with the oral antibiotic, the resolution of otitis media with effusion occurred on the 14 th day. In conclusion, the combination of nasal Azelastine and Fluticasone plus an oral antibiotic may be a safer and shorter therapy than monotherapy with an oral antibiotic in the treatment of serous otitis media, given the safety issues with long-term use of systemic antibiotics. Introduction: Beclomethasone dipropionate (BDP) nasal aerosol (nonaqueous) is approved for management of seasonal and perennial allergic rhinitis (SAR/PAR) in adolescents and adults. This study evaluated the efficacy and safety of 80 mg/d BDP nasal aerosol in children with PAR. Methods: A 12-wk, phase 3, randomized, double-blind, placebo-controlled, parallel-group, clinical trial was conducted at 62 US centers to investigate the efficacy and safety of BDP nasal aerosol in children (4-11 y of age) with PAR. A total of 547 patients were randomized (2:1) to receive 2 actuations of BDP nasal aerosol (1 actuation/nostril; 40 mg/actuation) or placebo nasal aerosol once daily. The primary end point was change from baseline in average morning (am) and evening (pm) reflective total nasal symptom score (rTNSS) over the first 6 wk of treatment in patients 6-11 y of age. Additional efficacy end points included change from baseline in average am and pm instantaneous TNSS (iTNSS) in children 6-11 y of age, and change from baseline in average rTNSS and iTNSS in children 4-11 y of age. Results: Improvement was significantly greater with BDP nasal aerosol (80 mg/day) than with placebo over the first 6 wk of treatment in children 6-11 y of age in average am and pm rTNSS and iTNSS (mean [95% CI] treatment difference: -0.66 [-1.08, -0.24], P = 0.002 and -0.58 [-0.99, -0.18], P = 0.004, respectively). Improvement in average am and pm rTNSS and iTNSS was significantly greater in patients 4-11 y of age receiving BDP nasal aerosol than in those receiving placebo over the first 6 wk of treatment (P = 0.002 and P = 0.004, respectively). Similar results were observed over 12 wk of treatment (P < 0.001). Improvement in average am and pm reflective individual nasal symptoms over the first 6 wk of treatment in 6-11 y olds was significantly greater for rhinorrhea (P = 0.004), nasal congestion (P = 0.001), and sneezing (P = 0.002) with BDP nasal aerosol than with placebo. BDP nasal aerosol was well-tolerated in children, with a safety profile comparable to placebo. Conclusions: This study showed that once-daily treatment with 80 mg BDP nasal aerosol (nonaqueous) in children 4-11 y of age was safe and effective in controlling nasal symptoms associated with PAR, suggesting that BDP nasal aerosol is effective for children with PAR. J. Kern *1 , L. Bielory 2 , S. Luster 2 , 1. Newark, NJ; 2. Springfield, NJ. Introduction: Allergic rhinitis is a prevalent condition impacting approximately 20% of the US population. There is a growing section of the population, up to 50% in some surveys, who are turning to alternative therapies for the treatment of their allergic rhinitis. Methods: A literature search was performed utilizing keywords rhinitis acupuncture, rhinitis traditional Chinese medicine (TCM), and rhinitis alternative therapy. Randomized control trials were reviewed in regards to their impact on symptom scores and objective equivalents and are displayed in table 1. Results: Decrease in total IgE: 39% from Yang et al. correlating with symptom improvement using various TCM formulations. 13.7% reduction in Zhang et al. Jung et al. showed a 3% increase in IgE levels in the group receiving TCM. Yang et al. demonstrated a 15% reduction in specific IgE to dust mites (p<.05). Acupuncture has been shown to have an impact on patient symptom scores as evidenced in table 1. In a randomized control trial by Brinkhaus et al. there was a minimal, however statistically significant, improvement in symptom score with acupuncture compared to sham therapy and medication alone. A follow up study comparing the combination of acupuncture with medication versus sham therapy with medication showed a reduction of 9% in the utilization of medication [p=0.01]. A large portion of studies were poorly constructed with significant bias given the operators' awareness of real versus sham therapy when applying acupuncture. Homeopathic therapy has been evaluated in several studies demonstrating mixed results in regards to its impact on allergic rhinitis. Several have found significant improvement. Taylor et al. demonstrated nasal airflow mean difference 19.8 L/min [p=0.0001], visual analogue score showed a symptom reduction of 28% in homeopathic group vs 3% reduction in placebo group [p=0.0007]. A larger study conducted by Aabel et al found no difference in symptom scoring from placebo. Conclusion: The evidence regarding the utilization of traditional Chinese medicine is mixed. Complementary therapy for allergic rhinitis demonstrates a potential benefit to patients in addition to current medical therapies. Alternative therapy studies revealed mixed results, however safety studies have shown minimal to no risk making them a safe addition to the treatment regime in select patients. Background: Mold sensitization is a risk factor for development and deterioration of upper airway allergy, especially chronic rhinosinusitis including those requiring surgery. Methods: 38 chronic sinusitis patients after surgery were included into the study. Routine medical examination, skin prick tests with common inhaled allergens an extended mold panel (Alternaria alternate, Cladosporium herbarium, Aspergilus fumigatus, Candida albicans, Mucor mucedo, Botrytis cinerea, Rhisopus nigricans, Penicilliumi notatum, Fusarum moniliforme Pullularia pullulans (Allergopharma, Germany) was done and IgE and allergen specific IgE measurement (Phadia, Sweden). All subjects were seen by an otolaryngologist and mycological examination was performed. Results: Sensitization to at least one allergen was present in 44.7% (17/38) of sinusitis patients. The most prevalent was sensitization to house dust mite Der-matophagoides pt., found in 21.0 % (8/38) patients. Positive skin prick tests with Candida albicans occurred in 18.4% (7/38), with Alternaria alternata in 18.4% (7/38), with Cladosporium herbarium in 10.52% (4/38), with Aspergilus fumigatus in 5.26 % (2/38), and with Botrytis cinerea in 2.63 % (1/38). None of these patients had sensitization to other mold allergens. Elevated allergen specific IgE was seen for Candida in 24% (9/38) patients, Alternaria alternata 26% (10/38), and Cladosporium herbarium in 8% (3/38). Conclusion: Nearly half of chronic sinusitis patients had sensitization to at least one allergen. Mold allergy was commonly seen in chronic sinusitis patients requiring surgery, being comparable in occurrence to dust mite sensitization. Background: The onset allergic diseases by age and gender may vary in childhood. The prevalence of various allergic diseases in patients stratified by gender into age groups who presented at the Allergy Outpatient Clinic of Western Hungary from 2002-2005 and from 2006-2009 were assessed. Methods: Only patients with newly diagnosed allergic diseases confirmed by skin prick tests and/or allergen specific IgE were assessed, being stratified by age into groups from newborn to 20 years old. From 2002 to 2005, there were 10,018 patients and from 2006 to 2009, there were 13,125 patients included in this epidemiologic study. Results: The total number of allergic patients increased from 0 through 5 years of age, then declined until adulthood in both investigated periods. There were more male patients under the age of 15, especially in the 5 year old age group in every year of the investigated period. After 15 years of age the female/male patient ratio increases rapidly. At 20 years of age, females are twice as likely to present with allergic diseases. Both the female and male patients in the age group analysis of various allergic diseases demonstrated the pattern of the "allergic march". Conclusion: Peak incidence of various allergic diseases occurred in the 5 years old age group. In early childhood the male/female ratio among allergic patients is high, this ratio being reversed in later adolescence. Patterns of prevalence of various childhood and adolescent allergic disease in Western Hungary demonstrated the progression from dermatologic to respiratory allergy characteristic of the allergic march. Background: The time interval from diagnosis of pseudotumor cerebri to development of CSF rhinorrhea is quite variable in literature. It can be intermittent and undetected for many years or may be the presenting symptom. We report a case of CSF rhinorrhea presenting three years after head trauma and pseudotumor cerebri. Methods: A 35-year-old woman with a five-year history of perennial allergic rhinitis to cat and mouse and moderate persistent asthma complained of intermittent persistent unilateral clear rhinorrhea, without any nasal congestion, sneezing or pruritus of six months duration. She denied any associated headache or wheezing. Results: Further review of systems was notable for history of head trauma three years prior to presentation, with development of pseudotumor cerebri, sinovenous thrombosis, papilledema, and macular degeneration. However, there was no skull fracture. Nasal examination was remarkable for swollen bilateral inferior turbinates. She was able to produce left sided nasal clear rhinorrhea by leaning forward. Fluid was tested for glucose and was found to be 307 mg/dL. She was promptly referred to neurosurgery. Confirmatory tests for new onset diabetes were performed given the degree of elevated glucose in the CSF. Discussion: Etiology of CSF rhinorrhea is classified as traumatic (>90%) or nontraumatic (<10%). Accidental head injury accounts for most traumatic cases, followed by iatrogenic etiology secondary to neurosurgical or rhinologic procedure. Nontraumatic causes are typically due to high intracranial pressure, tumors, erosive diseases, congenital defects of base of skull, or spontaneous without a specific etiology. Though our patient had no history of skull fracture to account for a CSF leak, she did have a history of pseudotumor cerebri after the trauma. While CSF rhinorrhea is a relatively rare entity in comparison to other common sinonasal inflammatory conditions such as the allergic rhinitis in our patient, this case illustrates the importance of maintaining a high index of clinical suspicion when encoun-tering a complaint of unilateral clear rhinorrhea with a remote history of trauma and/or increased intracranial pressure. This will ensure timely diagnosis and proper neurosurgical management that can ultimately prevent further complications such as bacterial meningitis or other serious intracranial infections. CHALLENGES OF CHRONIC COUGH. V. Ta *1 , K. Woessner 2 , 1. Redondo Beach, CA; 2. San Diego, CA. Introduction: Establishing gastroesophageal reflux disease (GERD) as the cause of chronic cough is challenging despite a careful history and initial evaluation because not all patients present with the usual symptoms of GERD. The term supraesophageal reflux disease (SERD) has been used to describe GERD with additional symptoms such as sore throat, hoarseness, globus, and excessive throat clearing. It should be emphasized that these laryngeal findings are not specific for SERD as a causative factor and the precise pathophysiologic mechanism responsible for the production of supraesophageal symptoms remains contentious. Case: The patient is a 60 year old male who presented with throat clearing and nonproductive chronic cough of one year duration. The cough occurs daily and is associated with the sensation of postnasal drip although he is unable to expectorate mucus. His symptoms are not positional or triggered by fumes or exercise. He denies food impaction or heartburn symptoms. The patient has tried various nasal corticosteroids, antihistamines, systemic corticosteroids and proton pump inhibitors (PPI) but these interventions failed to control his symptoms. His workup included negative skin prick, intradermal, and serum specific IgE testing to aeroallergens. His nasal cytology was negative for eosinophils. His CT sinus showed maxillary sinus mucosal thickening. He underwent an upper esophagogastro-duodenoscopy that showed no furrows, white patches, or ulcers. However, mid/proximal esophagus biopsy showed greater than 50 eosinophils on high power field. He also underwent a 24 hour pH probe which showed a supine pH average of 4.75. Discussion: Initial treatments include PPI and head of bed elevation even though acid suppressing medications have been shown to be only partially effective in treating SERD. Other options include surgery such as the Nissen fundoplication or the LINX System. Although 50 eosinophils/HPF was observed histologically, the patient denied any EoE symptoms and on these grounds the possibility of EoE was dismissed. The more likely explanation for this patient's cough is SERD, evident by his positive 24 hour pH probe. The patient tried and failed a short course of PPIs, however, a longer trial of a PPI is warranted. If ineffective, surgery will be considered. Spontaneous Chronic Urticaria (SCU ) is a common condition in adults and mainly among women, which coincides this phenomenon with autoimmunity. Our objective was assessed through laboratory studies whether patients with CUS presented autoimmunity data. Material and Methods: Cross-sectional study that 35 patients from the Allergy and Immunology at Juarez Hospital of Mexico diagnosed with CUS included, to which complete blood count CBC, thyroid antibodies (anti peroxidase and thyroglobulin), nuclear antibodies, rheumatoid factor, lupus anticoagulant and autologous serum skin test (ASST). Results: We evaluated 35 adult patients, 77.1% female and 22.9% male, ASST was positive in 11.4% patients, negative antinuclear antibodies in all cases but the antibodies anti thyroid (AAT) positive in 11.4% of LE cells, lupic anticoagulant and D dimer were negative in all patients and rheumatoid factor was positive in 1 patient. Positive patients AAT 100% were women and 75% of them had negative PSA. Tests of correlation between ASST and antibodies, was not significant. Conclusion: Patients with UCE most are women and only 11% had positive ASST but this does not correlate with anti thyroid antibodies or other common parameters of autoimmunity Background: Chronic urticaria (CU) is a benign, idiopathic and disturbing condition affecting a significant proportion of patients. A state of obesity has been linked with atopic diseases such as asthma. The association of obesity and CU has not been established. Methods: We performed a retrospective analysis of 86 Hispanic patients with CU visiting an Allergy and Immunology Clinic from June 2012 -August 2013 in Puerto Rico. Cases were stratified between physical urticaria (PU) and idiopathic urticaria (CIU). Demographic characteristics, comorbid diseases, and associated symptoms were compared. Data was analyzed using SPSS v19. Results: Subjects mean age was 32 ± 20 years, 77% were female. Concurrent atopic diseases were reported in 48% of patients, 31% had asthma. Most commonly associated symptom was angioedema (38%). Forty-four (51%) patients had CIU, while 41 (48%) had PU. 56% of the patients with PU reported more than one physical trigger. No significant associations were found between gender, thyroid disease, allergic rhinitis or food allergy and type of urticaria. Thyroid disease was more prevalent in adult participants (16/66; 24%), while allergic rhinitis was more common in adolescents and children (19/27; 70%). Patients with BMI > 25 were significantly more likely to have PU (20/31 (65%); p = 0.025), while subjects with a BMI <25 were two times more likely to suffer from CIU (29/49 (59%); p = 0.039). Conclusions: A significant association was found between increased BMI and PU compared to CIU. Studies are needed to evaluate the modulation of adipose tissue on mast cells and basophil degranulation. M.I. Rojo-Gutierrez * , C.N. Flores Ruvalcaba, J. Mellado Abrego, G. Castillo Narvaez, M. Gonzalez ibarra, Mexico City, DF, Mexico. Urticaria is a very common disease of skin, characterized by rapid onset of hives with or without angioedema. It is classified by etiology ; in spontaneous and inducible urticaria, and evolution in acute and chronic. IgE. CSU is more common in women and international management guidelines recommend 2nd generation antihistamines regulate dose or higher doses and in cases of failure suggest the use of monoclonal antibodies against IgE. Monoclonal antibodies are effective at doses of 150 and 300 mg so our aim was to determine their effectiveness at doses of 150mg and 300mg in patients with CSU. Materials and methods: We evaluated 35 patients who failed UCE in conventional antihistamine therapy .Two groups were randomly performed where the group : A) 20 patients received 150 mg single subcutaneous dose of omalizumab and B) 300mg 20 patients . Evaluate effectiveness by measuring frequency and intensity of the lesions with a baseline measurement UAS7 (Urticaria activity score 7 days) and weekly reviews and quality of life questionnaire (CUQ2oL) on arriving a week 1, 2 and 3. Results: we evaluated 40 patients 22.5 % (9) were male and 78.5 % (31) females. The mean was 226.93 CUQ2oL at baseline, 93.7 at the first week, 57 the second and the third 44.33. Hives/ pruritus in stockings 2.2/2.5 at startup, the first week 1.35/1.45, 0.9/1.1 second and 0.98 / 1.08 the third, we find highly significant differences when comparing home against 1st week, 2nd week and 3rd week p < 0.0001 . Conclusions: Omalizumab is highly effective in controlling symptoms of chronic spontaneous urticaria. V. Bozoghlanian * , D. Levy, R. Gutta, Irvine, CA. Introduction: Staphylococcus aureus is known to exacerbate atopic dermatitis with production of bacterial enterotoxins and superinfection of lesions. We report the first case of severe atopic dermatitis in a human exacerbated by Staphylococcus intermedius, which has been associated with exacerbation of atopic dermatitis in dogs. Case Description: A 26 year old Hispanic man presented with a 6-year history of extremely pruritic diffuse rash consistent with atopic dermatitis. He was referred for total IgE >16,000 IU/ml and failure to respond to high-dose topical and oral corticosteroids, methotrexate, cyclosporine, and phototherapy. In our clinic, an immunodeficiency evaluation was normal. Workup for hyper-IgE syndrome, including STAT-3 and Dock-8 gene mutations, was normal. Skin biopsies were negative for malignancy, fungal staining, or vasculitis, and suggestive of severe atopic dermatitis with normal immunofluorescence and immunophenotyping. Workup for a 70-pound unintentional weight loss was performed, with negative SPEP, UPEP, HIV, HTLV, bone marrow biopsy, flow cytometry, CT chest/abdomen/pelvis, endoscopies, and autoimmune evaluation. The patient had multiple severe exacerbations requiring several hospitalizations. Aggressive skin care measures (including wet/dry wraps), high-dose antihistamines, gabapentin, oral antibiotics, a onemonth trial of terbinafine, and a five-month trial of omalizumab were not efficacious. Multiple skin cultures identified highly-resistant Staphylococcus intermedius. The patient developed malignant otitis externa treated with daptomycin and meropenem. Cultures from the ear also grew Staphylococcus intermedius. His skin lesions improved on the IV antibiotics, but he continued to have a relapsing-remitting course when the antibiotics were withdrawn. Discussion: Staphyloccocus intermedius has been associated with exacerbation of atopic dermatitis in dogs. It may be transmitted to humans with close contact to dogs. In patients with severe atopic dermatitis refractory to first and second line therapies, it may be prudent to investigate for Staphyloccocus intermedius colonization and/or infection, and if identified, should be treated with tailored antibiotic therapy based on drug sensitivity. A. Hamad * , Madison Heights, MI. Introduction: Sweet syndrome has a presentation that is not uncommon; acute skin eruptions. It could be mistaken for many other conditions and expose patients to unnecessary medications and stress for months before reaching a diagnosis or worse delay a diagnosis of underlying malignancy. Case Description: Patient was a 52 year old female with past medical history of hypertension who was in her usual state of health until July 2013.She started having raised, painful, edematous and erythematous lesions. The tended to appear first in her hands and feet then rapidly spread to her forearms, legs and face. She occasionally had them in her chest. These lesions would appear every 7-8 days. They would last for 3-4 days then disappear, mostly spontaneously. She noticed that they were preceded almost always by fever. The patient initially went to the emergency room after the lesions first appeared. She was told that the lesions were likely hives and she was discharged home to follow up with her primary care physician. Her primary care physician switched her medication for hypertension (lisinopril) to another drug but that did not help. Later she was started on a short course of prednisone 40mg daily for 10 days. She also was prescribed topical corticosteroid and anesthetic creams. Patient did not have any relief with any of these interventions. Lesions continued to be a major concern for her. So, she was referred to a dermatologist and biopsy was performed for one of these lesions. The biopsy was consistent with "neutrophilic dermatitis". Patient later was referred to a hematologist/oncologist to rule out underlying malignancy. She was also referred to an Allergy-Immunology clinic where we had the chance to see her. She did not have active lesions at the time we saw her in the clinic but she showed us pictures she took for them at different sites of her skin. Our plan was to start the patient on colchicines as prednisone failed to show any improvement in her symptoms. Conclusion: It is crucial for general practitioners and allergists to keep sweet syndrome in the differential diagnosis of acute skin eruptions. As long as it is in the differential, the diagnosis can be confirmed with a skins biopsy. However, management can be as easy as discontinuing a causative drug or gets more complicated if there was an underlying malignancy. That's why it is important to increase awareness of this rare condition. T. Slavyanskaya *1 , V. Derkach 2 , B. Sangidorj 1 , 1. Moscow, Russian Federation; 2. Vladivostok, Russian Federation. The study was aimed at conducting a comparative cost-effectiveness (CE) analysis of various immunotropic therapy (IT) programmes of atopic dermatitis (AD) in children. 94 children at the age of from 3 to 18 with AD having lasted for 1-15 years were examined. All the patients received routine clinical and immuno-allergological examinations. The treatment was conducted in two stages: background therapy (BT) and IT. The patients were divided into three groups: the 1st group (n=30) received immunomodulator (IM) on a stepby-step basis in the course dose of 20 mg; the 2nd group (n=31) received an accelerated course of parenteral allergen-specific IT (PASIT); in the 3rd group (n=33) a combination IT (CIT) -IM+PASIT at an accelerated pace was applied. The CE comparative estimation of IT in the treatment of children with AD has shown the most optimal ratio in the 3rd group of patients receiving CIT. However, in the 1st and the 2nd groups in which IM and PASIT were applied, AD was not monitored well enough, that resulting from considerable expenses for hospital care. The ratio of costs for treating children with AD in hospital environment who received IM (the 1st group) was 42.95% of the total cost of AD treatment per year within direct expenses; the medicinal treatment was 48.12%. Among expenses for medications, 30.6% were for IM, and the other ones were associated with conducting BT for improving AD monitoring. In the 2nd group of children receiving PASIT, the principal item of direct expenses was the amount spent for visiting the allergologist for administering injections. The expenses for hospital care were 15.1%. In the 2nd group, the principal costs were associated with medicinal treatment of infectious complications. In the 3rd group of children receiving CIT, the percentage of direct expenses for hospital care was 8.92%, thus, being insignificant. Expenses for outpatient care (5.95%) and BT (8.95%) in this group were much lower than in the 1st and the 2nd groups due to the absence of infectious complications and reduction of BT volume. Inclusion of CIT in the integrated programme of therapy of children with AD resulted in reduction of BT volume, less admissions to hospital, prevention of secondary infection overlay; moreover, the disease was monitored efficiently at the same time. Thus, the CE ratio of the treatment was more rational. Introduction: Chronic idiopathic (spontaneous) urticaria (CIU) is a rare and symptomatic skin condition affecting patient health-related quality of life. Data on CIU-related healthcare resource use (HCRU) are old. We examined HCRU and costs in CIU patients using a healthcare claims database. Methods: We used a HIPAA-compliant fully de-identified commercial healthcare claims database from 2012 for this retrospective cohort study. We identified CIU patients with a previously validated ICD-9-CM coding algorithm. We examined patient characteristics, HCRU, and costs. Outcomes were stratified by demographics. We used chi-square tests and F-tests to conduct bivariate analyses. Because the study did not involve human subjects, IRB review was not needed. Results: The prevalence of CIU was 0.1% among all continuously enrolled patients. We identified 6,350 CIU patients with mean age 42.4 years, 68.3% women, and mean Charlson Comorbidity Index 0.9. CIU-related comorbidities included allergic rhinitis (43.2%), angioedema (23.5%), and asthma (18.3%). The most commonly prescribed drug classes were oral corticosteroids (OCS; 54.7%), antihistamines (24.0%), and leukotriene receptor antagonists (17.5%). Mean allcause total annual healthcare costs were $9,142 per CIU patient; outpatient services accounted for 43.0% of the costs. Per year, CIU patients averaged 15.1 physician office visits, 7.3% of patients had at least one hospitalization, and 15.9% -at least one emergency department (ED) visit. Total costs and HCRU increased significantly with age. Women accrued higher costs and had more hospitalizations and ED visits than men. CIU-specific annual costs totaled $997 per patient, and outpatient services accounted for about 2/3 of costs. Patients averaged 3.4 office visits for CIU-specific claims; 0.1% of CIU patients had at least one CIU-specific hospitalization and 1.9% at least one CIU-specific ED visit. Costs associated with CIU-based claims increased with age and were higher in women than in men; otherwise few consistent trends in HCRU emerged. Conclusions: Despite being otherwise relatively healthy, CIU patients had frequent HCRU, the majority of which were outpatient visits. CIU-specific HCRU was small compared with the total use, which also primarily comprised office visits. This indicates that CIU patients often see physicians for reasons not directly related to their urticaria. Introduction: Chronic urticaria (CU) has a prevalence of 30%. It is a condition that does not endanger life but has great impact on the quality of life. Sometimes, its management is difficult. Case 1: 33 year old female with urticaria for 1 year, and 2 months ago she has an exacerbation with the presence of edematous-erythematous, pruritic plaques, positive dermatographism and insomnia; eyelid edema and arms, she was treated with multiple antihistamines without success, we initiate methotrexate. We achieve a good control of symptoms. Case 2: 60 years old female, with urticaria for 3 years, which exacerbates in the past 3 months without responding to the multiple tratments, predominantly generalized pruritus and rash on both arms and abdomen; we indicate methotrexate with an good clinical response. Discussion: Urticaria management includes H1 antihistamines as the first option. Recent studies have reported the usefulness of methotrexate at a dose of 10-15mg per week, due to its anti-inflammatory effect rather than immunosuppression, proving to be effective in chronic idiopathic and autoimmune urticaria. Its mechanism of action is unclear, it increases adenosine levels, inducing apoptosis of activated CD4 lymphocytes and decreases neutrophil chemotaxis. It has an estimated response time of 3 weeks to 6 months. We report two cases of patients with urticaria with good response to methotrexate. It is a good option for patients with no response to antihistamines and other therapies. R. Patel *1 , G. Jyothirmayi 2 , E. Capitle 2 , 1. Long Island City, NY; 2. Newark, NJ. Introduction: Toxic Epidermal Necrolysis (TEN) is a dermatologic disorder characterized by diffuse erythema and necrosis of the epidermis. TEN can occur secondary to drugs, infections or autoimmune disorders (1). Methods: This is a 43 year old female,with a history of SLE maintained on hydroxychloroquine and azathioprine, hypertension and hypothyroidism, who presented with a progressively worsening erythematous and tender skin rash affecting her entire body. She was seen prior for a similar skin rash under the breast and discharged on cephalexin and fluconazole for possible cellulitis vs a fungal infection. She reported myalgias but denied any fever, vision changes, chest pain, shortness of breath, or genitourinary complaints. On physical examination, the patient was afebrile, with a blood pressure of 81/57, heart rate of 106, and a diffuse, erythematous, exudative, desquamating rash over 70 percent of her body including mucous membranes but no ocular involvement. Results: Further work up revealed leucocytois on CBC. The severity of illness score for TEN (SCORTEN) was 5 out 7 indicating a 90% mortality rate. Her acute symptoms were managed with intravenous (IV) fluids and broad spectrum antibiotics. Infectious work up was negative. Skin biopsy was indicative of TEN and she was treated with IV Immunoglobulin and steroids. Discussion: TEN is a life threatening skin disease and must be managed rapidly. This disorder belongs on a spectrum of skin diseases, which also involve Erythema Multiforme Major and Minor and Steven's Johnson Syndrome. History is an important aspect in the diagnosis of this condition. Details involving medication history, recent infections, and underlying autoimmune diseases must be thoroughly reviewed. In this patient, the rash was present prior to the start of antibiotic therapy, thus her underlying autoimmune disease of lupus most likely was the cause of TEN. Image 1: Our patient with a diffuse erythematous, exudative, and desquamating rash involving more than 70% of her body surface area. Introduction: Chronic idiopathic/spontaneous urticaria (CIU/CSU) symptoms interfere with patient sleep. Omalizumab has been shown to reduce disease activity in patients with CIU/CSU. We describe patient-reported data on sleep from GLACIAL -a large randomized double-blind placebo-controlled clinical trial. Methods: Subjects were randomized 1:3 and received placebo Introduction: Henna, derived from the plant Lawsonia inermis, has been used for centuries in India as a hair dye. In recent years, it has experienced resurgence, as consumers are increasingly drawn to natural products, including natural hair dyes. Interest in henna has also arisen as it is an exceedingly rare cause of allergy. Traditional hair dyes, on the other hand, make use of pphenylenediamine (PPDA). This is a common cause of allergic contact dermatitis, and is found in almost all permanent, semi-permanent, and demi-permanent hair dyes in the United States. Because of the extremely low risk of allergy, henna hair dyes are often recommended by physicians as appropriate alternate hair dyes for those allergic to PPDA. Case: A 50 year old male presented to dermatology clinic with a rash of the frontal hairline, eyebrows, and beard area. He reported that months earlier he had experienced an allergic reaction several days following the use of a drugstore brand of hair dye. His physician had diagnosed allergic contact dermatitis, likely due to p-phenylenediamine in the hair dye. He was instructed to use only henna hair dyes in the future. He then purchased a henna hair dye at an ethnic grocery store and applied it to his hair, eyebrows, and the beard area. Within three days he developed erythema, itching and swelling in each of these areas. A review of the ingredient list on the henna hair dye package revealed the presence of PPDA. Given his history, he declined patch testing. Conclusion: Henna is often recommended as an alternate hair dye in patients with PPDA allergy. For patients reporting PPDA allergy, henna has been recommended by physicians and hairstylists, as well as via multiple outlets on the internet. This case demonstrates that this is not always a safe strategy. Patients must be informed that some henna hair dyes may contain additional PPDA. This may be due to the fact that henna naturally produces a reddish tint to the hair. In order to produce a darker color, additional ingredients may be added in commercially available henna hair dyes; in this case PPDA. Patients must also be advised that packaging may not include dis-claimers, and therefore a review of the full ingredient list of any hair dyes is required prior to use. We report a case of a patiente with atopic dermatitis and allergic rhinitis with bad control with standard therapy. We added mycophenolate mofetil with good results, assessed by SCORAD scale. Case Presentation: Twenty-eight years old male from Veracruz. Since childhood, he has had pruritic erythematous scaly lesions scattered across the whole body surface, especially in the flexurar folds of the elbows, popliteal and ankle regions, creases of the neck and on the face; he also has extremely dry scaly skin. Symptoms have periods of remission and exacerbation. He also has hyaline anterior rhinorrhea, nasal obstruction, nasal pruritus and sneezing. He received moisturizers, topical and systemic steroids and calcineurin inhibitors without improvement. At initial assessment he has a SCORAD of 60. He has pruritic erythematous scaly papules with impetiginization. Studies show 9400 leukocytes, 3500 (38%) neutrophils, 2500 (27%) lymphocytes, 2400 (26%) eosinophils, 90 (1%) basophils. Blood chemistry, stool test, acute phase reactants (CRP and ESR), IgG, IgM, IgA and complement were normal. Total serum IgE 7830 IU /ml. Positive nasal eosinophils. Skin prick test was positive for Fraxinus and Dermatophagoides farinae. The diagnosis of severe atopic dermatitis and mild intermittent allergic rhinitis is confirmed by clinical and complementary studies. We started treatment for allergic rhinitis and atopic dermatitis based on intranasal saline solution, intranasal steroids, oral antihistamines, skin hydration, specific immunotherapy and added mycophenolate mofetil 1 g orally daily for 60 days. Clinical response of both allergic rhinitis and skin pathology was successful (SCORAD of 10). Discussion: Atopic dermatitis is a chronic inflammatory skin disease commonly associated with allergic rhinitis. The goals of treatment are to improve pruritus, papules, erythema, edema, vesicles and prevent impetigo and lichenification of the skin. Treatment was established with skin hydration, topical steroids, antihistamines and mycophenolate mofetil 1 g orally for 60 days. We conclude that mycophenolate mofetil is an effective treatment for difficult to control atopic dermatitis. Inflammatory skin diseases manifest via reactive physiological pathways to pathogens. Epidermal keratinocytes play a major role in skin inflammation. In acute eczematous dermatitis, activated dermis-infiltrating T cells secrete proinflammatory cytokines that direct epidermal keratinocytes to undergo either apoptosis or survival. Tumor necrosis factor may promote keratinocyte apoptosis and limit the spread of keratinocyte damage, leading to the formation of spongiosis, the histopathological hallmark of acute eczematous dermatitis. A 76 year old female presented to the allergy clinic with intermittent lesions in her left lower extremity. The patient had similar lesions in her chest and upper extremity in the past which resolved spontaneously. Her medical history is significant for rheumatoid arthritis, Sjogren's disease, fibromyalgia, breast cancer with bilateral mastectomy, gastroesophageal disease, myocardial infarction, neuropathy, depression, anxiety disorder, COPD, dyslipidemia, and hypothyroidism. Physical examination was significant for a 6.4cm discoid ulcer near the left ankle. RF, CCP, B cell markers, CD19, CD29, CD23, CD10, surface immunoglobulin, myeloid, platelet and natural killer cell, stem cell, erythroid cell markers and NBT were within normal limits. Allergen testing by sIgE was normal. Immunoglobulin A and M, as well as complement levels were normal; however immunoglobulin G levels were decreased. Biopsy of the area revealed spongiosis, epidermal hyperplasia, and a superficial perivascular and interstitial mixed inflammatory cell infiltrate. Sjögren's syndrome is a systemic autoimmune disease characterized by dry oral mucosa, dry eyes, and arthritis. Skin manifestations in Sjögren's syndrome occur in half of the affected patients. There are reports of atopic dermatitis complicated by Sjögren's syndrome and these patients present with persistent itchy dry skin and eczematous lesions. Perivascular infiltrates of lymphocytes, histiocytes, and occasional eosinophils can occur in dyshidrotic eczema in rheumatoid arthritis. Drug eruptions may present in virtually all patterns of cutaneous inflammation including spongiotic, lichenoid, and psoriasiform dermatitis. In psoriasiform dermatitis, the microenvironment of interacting inflammatory cells, antigen presenting cells, and epithelial cells is disturbed, leading to stereotyped reaction patterns in inflammatory skin diseases. Introduction: The chemical p-phenylenediamine (PPDA), found in almost all permanent hair dyes, is a common cause of allergic contact dermatitis. As the treatment of PPDA allergy centers on avoidance, the identification of a safe alternate hair dye is important. Henna is frequently recommended as an alternate, as allergy to henna is exceedingly rare. Derived from the plant Lawsonia inermis, henna has been used for centuries. This study was undertaken to evaluate commercially available henna hair dyes to determine their suitability as hair dyes in PPDA-allergic patients. Methods: A cross-section of local stores, including ethnic grocery stores, were surveyed in order to evaluate any henna hair dyes. Products were included if they contained the word "henna" in the name or on the package. The ingredient lists of all products were reviewed. Packaging was reviewed for any other indicators of PPDA presence. Results: A total of 6 stores were surveyed, and 25 brands of henna hair dyes identified. Of these, 21 contained the word "henna" in the name of the product, 2 used alternate words such as "mehandi" and 2 used the word "henna" elsewhere on the packaging. Eight of the 25 products lacked an ingredient list. Of the remaining 17 dyes, 7 listed PPDA as an ingredient and one contained disperse dyes. Only 3 of the 7 dyes containing PPDA provided disclaimers; the remainder displayed no notification of the presence of PPDA apart from the ingredient list. Conclusion: This analysis finds that multiple commercially available henna hair dyes could prove hazardous to patients with PPDA allergy. Almost half of labeled henna hair dyes in local stores contained PPDA, and the majority contained no notification of the presence of PPDA apart from the ingredient list. One additional product contained disperse dyes, a potential cross-reactor to PPDA. Another notable hazard was our finding that almost 1/3 of products contained no ingredient listing at all. This analysis makes clear that recommending henna hair dyes to patients with PPDA allergy is not sufficient. Many of the commercially available products would result in allergic reactions if used by those with PPDA allergy. Patients must therefore be advised to purchase only products that contain a full listing of ingredients, and to review the ingredients carefully prior to use. A. Kim * , San Diego, CA. Introduction: Acute generalized exanthematous pustulosis (AGEP) is a rare self-limiting drug eruption that classically presents with sterile pustules on an edematous and erythematous base. Diagnosing AGEP can be challenging due to potentially overlapping clinical features shared with septic shock, chronic blistering diseases, Stevens Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). Methods: Clinical case presentation. Results: A 49 year old woman was admitted to the intensive care unit for multi-organism urosepsis and started on vancomycin and ertapenem. Due to preexisting drug allergies, antimicrobial resistance and ertapenem-induced thrombocytopenia, Allergy/Immunology was consulted to facilitate Ceftriaxone desensitization. Prior to desensitization, the patient had stable hemodynamics and absent history of AGEP, DRESS, SJS/TEN, and visceral involvement from prior antibiotic administration. 2 hours following desensitization, the patient became febrile and tachycardic. Clinical deterioration progressed to profound hypotension overnight requiring initiation of two vasopressors. Infectious Disease evaluated the patient for urosepsis recurrence, but cultures were unremarkable. 96 hours following desensitization the patient developed erythema, edema, blistering, and desquamation on the dorsal feet with progression to her neck, perioral region, chest and upper back. Flaccid bullae and Nikolsky sign were present on the anterior thighs. Dermatology and Burn teams were emergently consulted and IVIG was initiated due to concerns of SJS/TEN. Skin biopsy revealed subcorneal neutrophilic pustular dermatitis with negative immunofluorescence. Blood analysis did not reveal eosinophilia or liver abnormalities and diagnosis of presumed AGEP secondary to Vancomycin was made. Conclusion: AGEP is a self-limiting and acute cutaneous eruption that typically presents with fever, pustules and erythema. Although AGEP generally has a clinical presentation distinct from SJS/TEN, there can be considerable clinical overlap necessitating care-ful histopathologic evaluation. As in our patient, AGEP can manifest with skin desquamation and positive Nikolsky sign, as well as profound disruption in hemodynamics. Given that the prognosis and management of AGEP is notably different from SJS/TEN, it is vital that physicians are aware of the diagnosis and its myriad clinical features. Introduction: It is important to evaluate the nutritional deficiencies in severely atopic infants in order to treat possible non-atopic causes of persistent dermatitis. We describe a case of acquired acrodermatitis enteropathica and endocrinopathy in an atopic child. Case Description: A full term 11 month old male with history of failure to thrive, GERD, and atopic dermatitis presented to our hospital with fever, dehydration, edema, and dermatitis. At 6 months of age, the patient's mother elected to feed with rice milk exclusively due to positive serum specific IgE testing to various foods. Our initial laboratory evaluation revealed anemia, lymphopenia, hypoalbuminemia, and transaminitis. His growth parameters were less than the 3 rd percentile, and he had anasarca. Skin exam revealed denuded, scaly, well demarcated erythematous plaques over the diaper area and erythematous crusted lesions around the mouth and eyes. The rash appeared 1 month prior to admission, and was not responsive to topical steroids. Skin biopsy was consistent with acrodermatitis enteropathica. Inpatient evaluation of his malnutrition further revealed markedly elevated thyroid stimulating hormone, and low serum zinc. An elemental diet and levothyroxine were started, and the patient's symptoms improved. Appropriate re-evaluation of his food hypersensitivity was performed and reintroduction of solids was successful. Discussion: This report highlights the potential morbidity of overly restrictive diets in children with severe atopic dermatitis. Patients with atopic dermatitis may have positive sensitization to various foods without history of clinical reaction. Foods used in lieu of infant formula may have varying levels of carbohydrates, fats, proteins, and minerals. These differences must be considered when dietary counseling takes place. Conclusion: Acquired acrodermatitis enteropathica should be considered in malnourished infants with steroid resistant dermatitis. Minerals are important co-factors in hormone production and function. Nutritionally appropriate alternatives should be recommended when discussing food elimination. Introduction:Treatment for urticarial rash could be frustrating if the conventional treatment fails but the side effect of medication seems to rise. We report a case of urticaria secondary to food allergy along with concomitant undiagnosed gluten-sensitive enteropathy.Methods: A 23 year old oriental female presented with 1 week history of the generalized urticaria including face and both palms. There were no GI symptoms at the initial presentation. She took a chitosan pill which is a product of shrimp and shell fishes as a food supplement prior to onset of the rash. There was a history of premenstrual pain which required high doses of advil. She had a frequent indigestion with unknown origin during chidhood. Initially,her urticarial rash responded well to IM steroid with IM Benadryl, followed by oral prednisone, hydroxyzine. But few days later, the patient started to have severe intermittent colicky abdominal pain with recurrent urticaria in spite of the treatment.The patient was seen at ER, started on hydrocodone-acetaminophen for pain without relief.Oral steroid was discontinued after the onset of the abdominal pain and changed to IM steroid which controlled urticaria but abdominal pain persisted. The patient was evaluated by two different gastroenterologists. The gastroendoscopy finding was negative. The blood test was positive for t-transglutaminase IgA: 5u/ml (0-3).The percutaneous skin test revealed multiple hypersensitivities to clam, shrimp, oyster, barley, celery, coconut, sweet potato, salmon, squash, strawberry, string bean, tomato, dust mites and chinese elm tree pollen but negative for wheat. Result:The gluten-free diet resulted in prompt remission of the recurrent colicky abdominal pain. The urticaria was under control with antihistamine along with elimination diets, allowing complete discontinuation of systemic steroids. Urticaria came back twice over 2 months in spite of gluten-free diet but subsided with antihistamines alone.Conclusion:The urticaria started with food supplemental pill which contained shrimp and shell fish proteins but undiagnosed gluten enteropathy became obvious during the treatment. The urticaria also can be a cutaneous manifestation of gluten-sensitive enteropathy aside from the food allergy. Therefore, the clinician should be aware of the role of concomitant underlying gluten-sensitive enteropathy in the treatment of urticaria. Introduction: Skin health, especially in atopic dermatitis (AD), relies on an intact skin barrier, a critical component of which is the protein filaggrin. If filaggrin production is impaired, skin barrier function is also dysfunctional and can lead to xerosis, infection, inflammation and allergic sensitization. Commensals may help support a healthy skin barrier by helping support normal filaggrin function. Using a patent-pending bio-extract from a novel probiotic human commensal organism, we examined whether the extract could be used to increase skin filaggrin protein levels. Methods: Using an ex vivo skin explant culture model with normal excised skin from plastic surgery patients, filaggrin induction by the proprietary bio-extract was tested via filaggrin antibody immunohistochemistry, rtPCR and ELISA. Normal skin explants were also used in a dry skin model induced by SDS treatment, with skin barrier integrity/hydration assessed via skin conductance; bio-extract effect was compared against dexamethasone. Results: Filaggrin immunohistochemistry: proprietary bio-extract at 0.05% and 0.1% increased filaggrin staining vs control. Filaggrin mRNA: filaggrin mRNA expression did not increase with bio-extract treatment at 0.25% and 0.1% vs control. Filaggrin ELISA: proprietary bioextracts at 0.05% and 0.1% increased filaggrin protein levels by over 2-fold vs control. Skin barrier function: after SDS treatment, 30 minute proprietary bioextract treatment increased skin conductance to or above normal levels; this improvement lasted for all time points measured and performed at least as well as dexamethasone. Conclusion: Patients look for "natural" disease treatment options but often fail to find effective choices. Even small increases in filaggrin have the potential to significantly improve AD clinical symptoms. The 2-fold increase in filaggrin protein levels accompanied by normalization of skin barrier function in human skin explants induced by a novel humanprobiotic derived bio-extract hold significant clinical promise in the treatment of skin diseases characterized by low filaggrin expression and skin barrier defects, such as AD. Introduction: It is increasingly recognized that maintenance of skin health, particularly in patients with atopic dermatitis (AD), requires homeostasis among the microbes comprising the dermatologic microbiome. Biofilms are the communal phenotype preferred by most microbes, including pathogens, and form on most surfaces. Commensals may help support a healthy skin barrier by inhibiting pathogenic biofilms such as MRSA. Using a patent-pending bioextract from a novel probiotic human commensal organism, we defined MRSA anti-biofilm activities of potential clinical utility in the prevention and treatment of MRSA biofilms. Methods: Using a 96-well biofilm microplate assay, proprietary bio-extracts were tested against clinical MRSA isolates for 2 antibiofilm activities: anti-biofilm adhesion and detachment of MRSA biofilm. Results were compared against vancomycin and meropenem. Results: Antiadhesion: The bio-extract was 2-3x more effective than antibiotic controls, with 35% anti-adhesion at 0.01 mg/ml, 50% at 0.05 mg/ml, 58% at 0.1 mg/ml, 85% at 0.5 mg/ml and 90% at 1 mg/ml when compared against untreated growth control. Biofilm detachment: The bio-extract was up to 10 times more effective than antibiotic controls: 0.05 mg/ml detached 50% of MRSA biofilm; vancomycin and meropenem both detached only approximately 5%. Unlike the anti-adhesive assay, there was no apparent dose-dependence, as percent detachment declined as bio-extract concentration increased. Lack of dose dependence may reflect non-metabolic mechanisms of action and/or brief application time. Conclusion: We have discovered a novel human-derived probiotic extract with anti-MRSA biofilm therapeutic effect. Skin of patients with AD is diffusely colonized by Staphylococcus aureus and MRSA. Increased microbial burden is associated with acute exacerbations, and chronically, these microbes are associated with inflammation, decreased skin immune defense and a weakened skin barrier. MRSA biofilms are a key factor in the persistence of these microbes in AD patients. This human probiotic-derived bio-extract with the dual antibiofilm activities of inhibition of biofilm adhesion as well as detachment of already formed pathogenic biofilm, could synergistically both treat and prevent MRSA biofilms in chronic skin diseases, such as AD. Rationale: Angioedema is a self-limited, localized swelling of the skin or mucosal tissues resulting from the extravasation of fluid into the interstitium. Although angioedema is a common condition in allergy practices, solar induced angioedema has a narrower differential diagnosis, including phototoxic drug reactions, solar urticaria, contact dermatitis, autoimmune inflammatory disorders and in rare cases metabolic disorders, such as cutaneous porphyrias. We present a case of erythropoietic protoporphyria (EPP) presenting as sun-induced angioedema in a 36 y/o Hispanic female. Methods: Case Report. Results: A 36 y/o female G1P1A0 presents to the clinic with burning, itching and swelling within minutes upon sun-exposed areas since her childhood. These episodes last several days, do not leave residual hyperpigmentation, but interfere significantly in her quality of life. Neither antihistamines nor sunscreens have been helpful to control her symptoms. Denies associated blisters, hives, hypertrichosis, anemia, exacerbation or provocation by any other physical stimuli, anemia, concomitant liver disease, family history of sun induced angioedema, nor NSAIDs use. Also denies gastrointestinal or respiratory complains or any comorbid conditions. Her family emigrated from Cuba and there is no history of intermarriage. Her only medication is vitamin D replacement. Past medical history was remarkable for chemical hepatitis after surgery for an ovarian cyst at 12 y/o and cholecystectomy for gallstones when she was 27 y/o. On physical exam a pale short size woman was noted without any visible eczema, hives, blisters or organomegaly. Initial laboratories were significant for mildly elevated liver function tests as well as elevated coproporphyrin and heptacarboxyl in urine. Anemia was not present. A preliminary diagnosis of EPP was made awaiting confirmatory porphyrin and protoporphyrin levels in RBC, which will be available for the presentation. Conclusion: Although EPP is a rare cause of sun-induced angioedema, it is important for allergists to consider it in the differential diagnosis in order to diagnose at an early age and prevent life-threatening complications such as hepatitis and hepatic failure requiring liver transplant, and to initiate appropriate sun avoidance recommendations. K. Dass * , A. Ditto, Chicago, IL. Elephantiasis nostras (EN) occurs secondary to a recurrent lymphangitis often introduced by trauma. Rarely, EN can affect the lips, with only four reported cases in literature. A 27 year old female presented to our allergyimmunology outpatient office with complaints of painful edema, erythema, peeling, and scabbing of her upper and lower lips. Her symptoms began 6 months prior to her visit. There was no history of drug allergy or prior history of angioedema. She denied any constitutional symptoms. Her symptoms were worse in the morning or when any food touched her lips. She denied any globus, tongue swelling, dyspnea. She denied any new medications or exposure to any new detergents or bathing products. Examination was notable for erythematous, edematous, painful lips with fissures at the vermillion border. There was no cervical or post-auricular lymphadenopathy. She was concerned about her appearance and lack of resolution of her symptoms. Elephantiasis nostras was considered as a diagnosis. The patient was treated with five days of prednisone and twenty-one days of amoxicillin clavulanate with complete resolution of her symptoms. Unfortunately, she was lost to follow up. With recurrence of symptoms, the patient followed with a dermatologist. She was treated for contact dermatitis despite avoiding all makeup or other skin products in the affected area. After testing positive for a nickel allergy, she also followed a low nickel diet. Despite her efforts, her symptoms persisted. . The patient returned to the allergy and immunology outpatient office, where she was successfully treated with a second course of 5 days of prednisone and 21 days of amoxicillin-clavulanate. Elephantiasis nostras can lead to permanent swelling, hypertrophic fibrosis, and facial disfigurement with repeated attacks. Suspicion of EN as a clinical entity may arise with unrelenting edema. Other disorders that should be considered include angioedema, contact dermatitis, erysipelas, granulomatous cheilitis, Melkersson's syndrome, superior vena cava syndrome, and scleroderma. Treatment focuses on long-term antibiotic administration with plastic surgery occasionally required. This case illustrates the importance of maintaining elephantiasis nostras as a differential diagnosis in patients with chronic edematous lips.